Approach To The Adult Patient With Splenomegaly and Other Splenic Disorders

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Approach to the adult patient with splenomegaly and other splenic disorders
Author: Stanley L Schrier, MD
Section Editor: William C Mentzer, MD
Deputy Editor: Jennifer S Tirnauer, MD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Nov 2016. | This topic last updated: Feb 25, 2016.
INTRODUCTION The spleen is a hematopoietic organ capable of supporting elements of the erythroid,
myeloid, megakaryocytic, lymphoid, and monocyte-macrophage (ie, reticuloendothelial) systems [1]. As such,
it is important in the following situations:
The spleen participates in cellular and humoral immunity through its lymphoid elements. (See "The
adaptive cellular immune response" and "The humoral immune response".)
The spleen is involved with the removal of senescent red blood cells, bacteria, and other particulates
from the circulation through elements of the monocyte-macrophage system. An increase in this function
(ie, hypersplenism) may be associated with varying degrees of cytopenia, while removal of the spleen (ie,
asplenia) may render the patient susceptible to bacterial sepsis, especially with encapsulated organisms
[2]. (See "Extrinsic nonimmune hemolytic anemia due to mechanical damage: Fragmentation hemolysis
and hypersplenism", section on 'Extravascular nonimmune hemolysis due to hypersplenism' and "Clinical
features and management of sepsis in the asplenic patient" and "Prevention of sepsis in the asplenic
patient".)
Splenectomy in patients with various hematologic disorders (eg, thalassemia, stomatocytosis) has been
associated with an increased incidence of vascular complications, including venous and arterial
thrombosis and pulmonary hypertension [2,3].
Normally, approximately one-third of circulating platelets are sequestered in the spleen, where they are in
equilibrium with circulating platelets.
Under abnormal circumstances (eg, primary myelofibrosis), the spleen may become the site of
extramedullary hematopoiesis and contain developing erythroid, myeloid, and megakaryocytic
precursors. (See "Clinical manifestations and diagnosis of primary myelofibrosis", section on
'Extramedullary hematopoiesis'.)
This topic review will discuss the approach to a patient whose spleen is enlarged on physical examination (ie,
splenomegaly) or is more than minimally enlarged on ultrasound, x-ray, nuclear medicine liver-spleen colloid
study, CT scan, or magnetic resonance imaging. However, it should be remembered that the diagnostic
criteria for various disease states have historically been built on long experience with the physical
examination, while scanning procedures have been available for a shorter period of time. Thus, the clinical or
diagnostic significance of a spleen that is modestly enlarged on scan but is not palpable (ie, "scanomegaly")
is uncertain.
As an example, in polycythemia vera, palpable splenomegaly was a major diagnostic criterion according to
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the Polycythemia Vera Study Group. However, a spleen enlarged on a scanning procedure has been
considered to be only a minor criterion using other diagnostic algorithms. (See "Clinical manifestations and
diagnosis of polycythemia vera".)
The approach to the child with splenomegaly is discussed separately. (See "Approach to the child with an
enlarged spleen".)
SPLENIC DISORDERS
Normal splenic function The spleen lies within the peritoneal cavity in the posterior portion of the left
upper quadrant, below the diaphragm and adjacent to the 9th to the 11th ribs, stomach, colon, and left kidney
(figure 1), with its hilum in close approximation to the tail of the pancreas (figure 2). The spleen weighs 80 to
200 grams and 70 to 180 grams in the normal adult male and female, respectively, averaging about 150
grams, or approximately 0.2 percent of body weight [4]. It is not usually palpable, but may be felt in children,
adolescents, and some adults, especially those of asthenic build.
The spleen is a major lymphopoietic organ, containing approximately 25 percent of the total lymphoid mass of
the body; this component, as well as components of the monocyte-macrophage system, can react and
enlarge quickly after the onset of infection or inflammation. Under such circumstances, a spleen enlarged to
scan but not palpably enlarged may be a nonspecific marker of inflammation similar to an elevated
erythrocyte sedimentation rate or other acute phase reactants. (See "Acute phase reactants".)
A major function of the spleen is to remove particulates (eg, opsonized bacteria, antibody-coated cells) from
the blood stream [5]. This function is most apparent when the spleen has been removed, since
splenectomized patients are susceptible to bacterial sepsis, especially with encapsulated organisms. This
function is also associated with trapping and destruction antibody-coated platelets or red cells in patients with
immune thrombocytopenia (ITP) or autoimmune hemolytic anemia, respectively. (See "Clinical features and
management of sepsis in the asplenic patient", section on 'Role of the spleen in host defense' and "Immune
thrombocytopenia (ITP) in adults: Clinical manifestations and diagnosis", section on 'Pathogenesis' and
"Warm autoimmune hemolytic anemia: Treatment", section on 'Splenectomy'.)
The spleen also serves as a quality control mechanism for red cells, removing senescent and/or poorly
deformable red cells from the circulation. This "culling" function is taken advantage of when splenectomy is
employed as treatment for hereditary spherocytosis. (See "Hereditary spherocytosis: Clinical features,
diagnosis, and treatment", section on 'Splenectomy'.)
As part of this quality control function, the spleen also removes particles from within circulating red cells (ie, its
"pitting" function), such as nuclear remnants (Howell-Jolly bodies), insoluble globin precipitates (Heinz
bodies), and normally-occurring endocytic vacuoles. Of clinical importance, Howell-Jolly bodies appear in
circulating red cells when the spleen has been surgically removed or has reduced function (ie, hyposplenism),
and subsequently disappear when and if splenic function returns, as in the following circumstances:
Splenosis due to the growth of splenic implants resulting from the spillage of cells from the splenic pulp
during splenectomy or following trauma to the abdomen. (See 'Splenosis' below.)
Growth of preexisting accessory spleen(s) or splenic implants following splenectomy, classically (albeit
rarely) described in patients with immune thrombocytopenia (ITP) or autoimmune hemolytic anemia
(AIHA) undergoing a late relapse after successful splenectomy [6,7].
Improvement in splenic function, as seen in some patients with sickle cell anemia following successful
allogeneic hematopoietic cell transplantation [8] or institution of a chronic transfusion program in children
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or young adults [9,10].

Hypersplenism Normally, about one-third of the platelet mass is sequestered in the spleen, where it is in
equilibrium with circulating platelets. Splenic sequestration of platelets can be increased to 90 percent in
cases of extreme splenomegaly, although total platelet mass and overall platelet survival remain relatively
normal (figure 3) [11].
Patients with cirrhosis, portal hypertension, and splenomegaly may have significant degrees of "apparent"
thrombocytopenia (with or without leukopenia and anemia), but rarely have clinical bleeding, since their total
platelet mass is usually normal. (See "Extrinsic nonimmune hemolytic anemia due to mechanical damage:
Fragmentation hemolysis and hypersplenism", section on 'Extravascular nonimmune hemolysis due to
hypersplenism'.)
Hyposplenism and asplenia Splenic functions are lost when the spleen is congenitally absent [12], has
been surgically removed, has atrophied following repeated infarction (eg, sickle cell disease), or following
splenic artery thrombosis.
Splenic function is reduced in the neonate and may be abnormally reduced (called hyposplenism or functional
asplenia) when it is engorged with blood (eg, splenic sequestration crisis associated with sickle cell disease,
malaria, splenic vein thrombosis), or infiltrated (eg, sarcoidosis, amyloidosis, tumors, cysts) [13-15].
Functional asplenia has also been described in a number of conditions, including autoimmune disease,
severe celiac disease, inflammatory bowel disease, chronic graft-versus-host disease, and untreated HIV
infection; the mechanism in most of these latter settings is unclear [16-19]. (See "Pathogenesis, epidemiology,
and clinical manifestations of celiac disease in adults", section on 'Hyposplenism'.)
Subjects with hyposplenism or functional/surgical asplenia may show any or all of the following [19-21] (see
"Overview of the clinical manifestations of sickle cell disease", section on 'Infection'):
Sepsis with encapsulated organisms, often fatal, especially in children. Management of such patients for
both the prevention and treatment of bacterial sepsis is discussed separately. (See "Prevention of sepsis
in the asplenic patient" and "Clinical features and management of sepsis in the asplenic patient", section
on 'Incidence'.)
Mild degrees of thrombocytosis (table 1) and leukocytosis.
The presence of Howell-Jolly bodies in circulating red cells, increased numbers of target cells and
misshapen red cells (picture 1 and picture 2), and red cells with reduced deformability [22]. Howell-Jolly
bodies can be quantitated to provide an estimate of the degree of splenic hypofunction [23,24]. (See
"Evaluation of the peripheral blood smear", section on 'Howell Jolly bodies'.)
Increased number of erythrocyte "pits" and Heinz bodies. Demonstration of these abnormalities requires
special techniques such as interference microscopy and supravital dyes, respectively. Quantitation of the
number of "pitted" erythrocytes has been used to estimate the volume and function of splenic tissue
remaining after splenectomy [25]. As an example, in one study in children, the mean proportion of "pitted"
red cells was 0.08 percent (range 0 to 0.8 percent) in normal controls with intact spleens, 20.3 percent
(range 12 to 33 percent) in those whose spleens had been removed for various hematologic or oncologic
indications, and 2.8 percent (range <1 to 7 percent) in those who had evidence of residual splenic activity
(splenosis) following splenectomy for trauma [26]. (See 'Splenosis' below.)
Depending upon the degree of hypofunction, imaging studies may demonstrate decreased or absent
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uptake of radioactive colloid, red cells, white cells, and/or platelets by the spleen [13-15,17,19].
Splenosis Implants of splenic tissue may result from the spillage of cells from the splenic pulp following
abdominal trauma, accidental lesions to the spleen during operation, or elective splenectomy, a condition
called splenosis. Estimates of the frequency of splenosis following such events vary widely, in the range of 26
to 65 percent following trauma, and 16 to 20 percent following elective splenectomy for hematologic disorders
[26-30].
Splenic implants, which are often multiple, can be located anywhere in the peritoneal cavity, although
they are most often found in the left upper quadrant of the abdomen (image 1). They may be confused
with neoplasms or endometriosis [31-33], and may rarely be the cause of small bowel obstruction (image
2) [34,35] or hemorrhage [36].
If traumatic splenic rupture is accompanied by a diaphragmatic tear, implants can also be present within
the pleural or pericardial cavities [27,37-39] or elsewhere in the chest [40]. (See "Surgical management
of splenic injury in the adult trauma patient", section on 'Splenosis'.)
These implants, which consist of normal splenic tissue, can be diagnosed via tissue biopsy techniques [40], or
preferably non-invasively through radioisotope scanning, using either Tc99m sulfur colloid or labeled platelets
or heat-denatured red cells [41-43].
If the patient has had a prior elective or emergency splenectomy, a presumptive diagnosis of the presence of
splenic implants/splenosis can be made if the peripheral smear no longer shows the presence of Howell-Jolly
bodies or the percent of "pitted" red blood cells is found to be low (see 'Hyposplenism and asplenia' above)
[39].
However, return of normal overall splenic function in such cases is usually incomplete [29]. In one follow-up
study in 90 patients whose spleens had been removed either because of trauma or as an elective procedure,
a high inverse correlation was found between "pitted" red cell counts and computed residual splenic volume
[25]. All patients with "pitted" red cell values less than 16.2 percent had residual splenic tissue visualized on
scintigraphy. When measured, their computed splenic tissue volumes were in the range of 22 to 133 cm3,
suggesting that splenic function is minimal when the volume of splenic implants is less than 20 cm3. (See
'Hyposplenism and asplenia' above.)
Splenic abscess Splenic abscess is an uncommon infection that typically results from endocarditis or
seeding from some other site of infection [44-48]. The frequency of this complication was evaluated in a
review of 564 patients with documented endocarditis in whom 27 (4.8 percent) developed splenic abscess
[45].
Typical clinical manifestations are fever that may be recurrent or persistent despite antimicrobial therapy and
left upper quadrant pain with or without splenomegaly [45,46,48]. However, some patients lack these classic
features [49]. Splenic abscess may be accompanied by a left-sided pleural effusion [48] or by splenic
infarction if due to septic emboli [44].
A splenic abscess can usually be seen on CT scan [44,45], although the lesion may be incorrectly called an
infarct [45] which also can be a complication of bacterial endocarditis [44]. (See 'Symptoms' below.)
Splenic abscess is usually managed by a combination of antibiotic therapy and splenectomy [44-46,49].
CT-guided percutaneous aspiration is occasionally successful, but this approach has not replaced
splenectomy as the standard of care [49].
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Splenic infarction Splenic infarction occurs when the splenic artery or one or more of its sub-branches
become occluded with an infected or bland embolus or clot. Affected patients classically present with acute
left upper quadrant pain and tenderness, although atypical presentations are common. As an example, in a
study of 26 patients with splenic infarction seen at a single medical center, the following clinical and laboratory
features were noted [50]:
Left-sided abdominal pain: 48 percent; abdominal pain was absent in 16 percent
Fever >38C: 36 percent
Left upper quadrant abdominal tenderness: 36 percent; abdominal tenderness was absent in 32 percent
Nausea or vomiting: 32 percent
Splenomegaly: 32 percent
Elevated serum lactate dehydrogenase (LDH): 71 percent
White blood cell count >12,000/microL: 56 percent
Others have described fever, unexplained pleural infusion, and/or splenic or peritoneal friction rub as initial
manifestations of a septic splenic infarction [50,51].
Splenic infarction can occur in a variety of settings [52]:
Presence of a hypercoagulable state (eg, malignancy, antiphospholipid syndrome) [53]
Embolic disease (eg, atrial fibrillation, patent foramen ovale, atheromatous disease, infective
endocarditis) [44,54,55]. Splenic artery embolization has been therapeutically employed in order to
reduce splenic blood flow in some patients with severe portal hypertension, prior to laparoscopic
splenectomy in order to diminish intraoperative blood loss, as well as for the treatment of splenic injury
[56]. (See "Ascites in adults with cirrhosis: Diuretic-resistant ascites", section on 'Splenic artery
embolization' and "Hereditary spherocytosis: Clinical features, diagnosis, and treatment", section on
'Splenectomy'.)
Underlying myeloproliferative neoplasm with associated (often massive) splenomegaly (eg, primary
myelofibrosis, post-polycythemia vera myelofibrosis)
Underlying hemoglobinopathy, especially sickle cell disease [57-59]. (See "Hepatic manifestations of
sickle cell disease", section on 'Computed tomography' and 'Hyposplenism and asplenia' above.)
Any condition associated with marked splenomegaly (eg, Gaucher disease, splenic lymphoma). (See
"Gaucher disease: Pathogenesis, clinical manifestations, and diagnosis", section on 'Visceral disease'.)
Trauma to the spleen which compromises its vascularity. Splenic hematoma and laceration may also
occur following blunt abdominal trauma. This subject is discussed in detail separately. (See "Liver,
spleen, and pancreas injury in children with blunt abdominal trauma" and "Liver, spleen, and pancreas
injury in children with blunt abdominal trauma", section on 'Spleen'.)
Splenic arterial torsion, as seen in the "wandering spleen" syndrome. (See "Causes of abdominal pain in
adults", section on 'Less common causes'.)
Splenic infarction and splenic rupture are uncommon complications of infectious mononucleosis. (See
"Clinical manifestations and treatment of Epstein-Barr virus infection", section on 'Splenic rupture'.)
Treatment of splenic infarction depends upon the underlying cause. Simple cases may require only
medication for relief of local pain and other forms of supportive care, while complications such as splenic
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abscess or rupture may require surgical intervention.

Splenic calcification and cysts With the increase in ultrasound and imaging procedures, patients are
being referred who have abnormalities in splenic architecture that frequently have nothing to do with the
patient's complaints. This has led to patient anxiety and multiple extensive and repetitive further imaging
studies at considerable expense and little apparent clinical benefit. Two such findings are splenic calcification
and splenic cysts.
Calcification Splenic calcification may be noted on conventional imaging techniques, and may or may
not be associated with splenomegaly. A vast number of abnormal conditions may be associated with such
calcifications, including phleboliths, splenic artery aneurysm, sickle cell disease [60], tumors (eg,
hemangioma, hemangiosarcoma, lymphoma), and infections (eg, histoplasmosis, brucellosis, echinococcosis,
candidiasis, tuberculosis) [61-69].
Gamna-Gandy bodies are fibro-siderotic nodules impregnated with iron pigment (hemosiderin) and calcium
that may appear in the spleen in congestive splenomegaly, sickle cell anemia, and hemochromatosis [70].
They likely represent areas of organized hemorrhage.
Cysts and pseudocysts A splenic cyst (or multiple cysts) may be noted as an incidental finding on
conventional imaging techniques, or as a result of evaluation of a patient with left upper quadrant pain, left
shoulder pain, abdominal enlargement, or splenomegaly. A vast number of abnormal conditions, many of
which are rare, may be associated with such cysts, including [71-73]:
Post-traumatic cysts/pseudocysts, including cystic splenosis
Hydatid (echinococcal) cysts
Congenital cysts
Epidermoid, mesothelial cysts
Hemangioma, lymphangioma
Polycystic kidney disease with splenic cysts
Splenic peliosis
Cystic metastasis to the spleen
Some splenic cysts may remain unchanged for many years, while others may enlarge slowly, enlarge to
massive proportions, rupture, bleed, or become secondarily infected. Since non-parasitic splenic cysts are
rare, there is no evidence-based information regarding their optimal surgical management [74].
For those with symptomatic cysts, or cysts that are enlarging over time, a number of radiologic and surgical
procedures are available for investigating the probable diagnosis [74-76]. Available options for those with
non-parasitic cysts include percutaneous procedures (eg, biopsy, aspiration, drainage), or more direct surgical
interventions such as decapsulation/cyst wall unroofing, partial or total splenectomy. However, only
splenectomy provides diagnostic certainty, which is rarely clinically justified.
Treatment of echinococcal cysts is discussed separately. (See "Treatment of echinococcosis".)
Splenic rupture
Traumatic splenic rupture Blunt abdominal trauma most often results in injury to the spleen, which in
over 60 percent of cases is the only damaged intraperitoneal structure. Delayed splenic rupture can occur.
This subject is discussed separately.
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(See "Initial evaluation and management of blunt abdominal trauma in adults".)

(See "Management of splenic injury in the adult trauma patient".)
(See "Surgical management of splenic injury in the adult trauma patient".)
(See "Liver, spleen, and pancreas injury in children with blunt abdominal trauma", section on 'Spleen'.)
Atraumatic splenic rupture Splenic rupture in the absence of trauma is uncommon, but may be
life-threatening [77]. In a systematic review of 845 cases from the literature, in which the mortality was 12
percent, six major causes were described [78]:
Neoplasm (eg, leukemia, lymphoma) 30 percent
Infection (eg, infectious mononucleosis, CMV, HIV, endocarditis, malaria) 27 percent
Inflammatory disease/non-infectious disorders (eg, acute and chronic pancreatitis, primary amyloidosis)
20 percent
Drug and treatment related (eg, anticoagulation, G-CSF, thrombolytic therapy, dialysis) 9 percent
Mechanical causes (eg, pregnancy-related, congestive splenomegaly) 7 percent
Idiopathic (normal spleen) 7 percent
Risk factors for mortality included splenomegaly, age >40 years, and the presence of a neoplastic disorder.
Splenectomy was performed in 84 percent of the cases, with conservative measures taken in the remainder.
Splenic artery aneurysm Splenic artery aneurysms are the third most common aneurysms found within
the abdomen, after those of the aorta and iliac arteries. They are clinically important because of the possibility
of rupture, which is associated with a high mortality rate, especially during pregnancy and/or in patients with
portal hypertension [79,80]. They are often calcified and may be mistaken for lesions of the distal pancreas
(image 3) [81]. (See "Pregnancy in women with pre-existing chronic liver disease", section on 'Cirrhosis and
portal hypertension' and "Approach to abdominal pain and the acute abdomen in pregnant and postpartum
women", section on 'Disorders of the spleen'.)
Treatment options include surgical resection with vascular reconstruction, stenting of the vessel or
endovascular coil/glue ablation techniques [82-84].
Symptoms The presence or absence of symptoms due to an enlarged spleen depends on many factors,
such as the acuteness and nature of the underlying illness, as well as the size of the spleen. Thus, a
minimally enlarged spleen secondary to an acute viral infection may be quite tender, while a markedly
enlarged spleen in one of the chronic myeloproliferative disorders (eg, polycythemia vera, primary
myelofibrosis) may be totally asymptomatic unless there is an episode of splenic infarction.
When present, symptoms of an enlarged spleen may include one or all of the following:
Pain, a sense of fullness, or discomfort in the left upper quadrant
Pain referred to the left shoulder
Pleuritic pain
Early satiety, due to encroachment on the adjacent stomach
Acute pleuritic-like pain and tenderness in the left upper quadrant in the presence of fever suggests the
presence of perisplenitis or splenic abscess, most likely due to infection originating elsewhere in the body (eg,
sepsis, infective endocarditis). Splenic abscess may be accompanied by a left pleural effusion [48] or by
splenic infarction if due to septic emboli [44]. Some patients with splenic infarction have an associated friction
rub over the infarcted area. (See 'Splenic disorders' above and "Causes of abdominal pain in adults".)
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SPLENIC SIZE AND PALPABILITY The median splenic weight in adults is about 150 grams [4,85]. It is
not usually palpable, but may be felt in children, adolescents, and some adults, especially those of asthenic
build [86]. A spleen becomes palpable not only as a result of its size, but also its texture. Ordinarily, the
spleen is a soft organ, but a spleen infiltrated with lymphoma or one with extramedullary hematopoiesis (eg, a
myeloproliferative disorder) is much firmer and thus easier to feel. (See 'Normal splenic function' above.)
Physical examination A number of studies have shown wide interobserver variability in the ability to
appreciate an enlarged spleen, which is generally not associated with the level of clinical experience [87].
There are at least six different palpation and percussion maneuvers available. Only two of these will be
discussed here: abdominal palpation and percussion of Traube's semilunar space. The interested reader is
referred to the literature and textbooks of physical examination techniques for further information on this
subject [55,87-89].
Palpation method The most frequent errors made in examination of the spleen involve incomplete
relaxation of the abdominal musculature of the patient and the musculature of the examiner's hand(s).
Effectiveness in palpating the spleen can be maximized by remembering that the major error in splenic
palpation is due to pressing too hard on the patient's abdomen, and by paying attention to the following:
With the patient supine, allow the patient to feel the examining hand on the abdomen before pressing
down, and to become adjusted to its presence. Do not suddenly increase pressure during palpation, as
an enlarged spleen may be quite tender (particularly if it has enlarged quickly) and the patient may be
reluctant to allow the examination to continue.
Make sure that the patient is relaxed, with arms at the sides of the abdomen. If the arms are raised, this
may stiffen the abdominal musculature and make examination more difficult.
Relaxation of the patient can be improved if the legs and neck are slightly flexed. Relaxation of the
examiner can be improved by being comfortably seated in a chair alongside the patient's bed or
examining table, with the examiner on the patient's right side, the right hand doing the palpation and the
left hand underneath and supporting the patient's left lower posterior rib cage.
A spleen that is only minimally enlarged will be quite movable with respiration, and may be palpable only at
the end of inspiration. Using a light touch, with the skin depressed under the left costal margin, a minimally
enlarged spleen can be felt as a rounded edge with the consistency of normal liver, which slips under the
examiner's fingers at the end of inspiration and back on expiration.
Since the spleen is normally a posterior structure, increased sensitivity can occasionally be obtained by
placing the patient in the right lateral decubitus position, with knees and neck flexed. This maneuver also
increases relaxation of the abdominal musculature and rotates the spleen to a more anterior position. One
study suggested that this additional maneuver was not useful when it followed examination of the patient in
the supine position [55]. However, it is this authors' experience that some spleens not palpable with the
patient in the supine position are felt with the patient in the right lateral decubitus position.
With greater degrees of enlargement, the spleen rotates to a more anterior and rightward position, and may
extend downward into the pelvis. Under these circumstances, the lower pole of the spleen may not be easily
felt (since it is well below the left costal margin), and splenomegaly is appreciated either by palpating at
successively lower levels on the left side of the abdomen or by palpating the medial edge of the spleen. The
presence of a notch or indentation on the medial splenic edge is a further indication that the mass is spleen
and not the left kidney or a pancreatic pseudocyst.
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In more extreme cases, the enlarged spleen extends across the midline, and may even be palpable in the
right upper quadrant. The presence of exquisite splenic tenderness suggests the presence of infarction or
perisplenitis in such massively enlarged spleens.
Percussion of Traube's semilunar space The tympanitic area overlying the gastric stomach bubble in
the left lateral hemithorax has been called Traube's semilunar space [90]. The size and location of this space
depends upon the contents and position of the stomach. The space will be obliterated in the presence of left
pleural or pericardial effusion, and will be displaced downward following enlargement of the left lobe of the
liver or, more commonly, the spleen. False-positive and false-negative results may be obtained, however, in
patients examined too soon after a meal and in obese subjects, respectively [91].
Accuracy The ability to palpate an enlarged spleen depends upon several variables, including:
The size of the spleen. Minimally enlarged spleens may not be felt under any circumstance. In one study,
all spleens with an estimated weight (from scanning studies) exceeding 300 grams were palpable, with
the average estimated weight of a palpable spleen being 285 grams [92]. However, some spleens
weighing as much as 900 grams are not palpable [93].
The body habitus of the patient. The spleen is easier to feel in thin individuals and in those who do not
have an increased anterior-posterior thoracic diameter.
The skill of the examiner coupled with the ability of the patient to cooperate during the examination.
The method(s) employed. (See 'Splenic imaging for size estimation' below.)
Using ultrasound examination of the spleen as a gold standard (criteria described above), a number of
studies have compared the various palpation and percussion techniques for evaluating splenic size. Major
conclusions from all of these studies are the insensitivity of available techniques, wide interobserver
variability, and the complementary value of combinations of techniques (ie, palpation plus percussion).
Specific observations in individual studies can be summarized:
Palpation of the spleen was significantly more accurate in lean versus obese individuals [55]
Sensitivity and specificity of percussion of Traube's semilunar space were 62 and 72 percent,
respectively [91].
The specificity of direct splenic palpation was 92 percent, with a positive predictive value of 92 percent
[89].
The combination of positive results on percussion of Traube's space (ie, dullness to percussion) and
positive palpation had a sensitivity and specificity of 46 and 97 percent, respectively [55].
If palpation maneuvers convince the examiner that the spleen is enlarged, this high degree of specificity
suggests that follow-up scanning to confirm the finding is not necessary. However, scanning may still be
useful to document baseline splenic involvement and size in preparation for treatment, or to document the
presence of other abnormalities (eg, infiltrative disease of other abdominal organs, intraabdominal
lymphadenopathy).
Splenic imaging for size estimation There is a reasonably close correlation between splenic weight and
splenic size on external scanning. This was illustrated in a retrospective study of 81 patients who had
undergone a total of 101 abdominal ultrasound examinations within four months of death and whose spleens
were weighed during autopsy; the splenic weight in grams was equal to (0.43 x length x width x thickness) for
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measurements in centimeters [94].

A number of criteria have been proposed to define the size of the normal spleen in adults using these
procedures:
Ultrasound The spleen is considered to be normal in size if its length is <13 cm [87] or its thickness is
5 cm on ultrasound examination [95]. In one study of healthy stem cell donors, mean values for splenic
length and width were 10.8 and 3.6 cm, respectively [96]. The mean predictive errors for repeated
ultrasound measurements of splenic length and width were 1.5 and 1.9 mm, respectively.
Plain film The spleen is normal in size if it is not seen on the abdominal plain film, if it is <5 cm in
width, or if it is less than 85 percent of the size of the normal kidney [92]. It is considered enlarged if it is
>6 cm wide or >13.6 cm long, or if (length x width) is >75 cm2.
Liver-spleen colloid scanning The spleen is normal in size if its length on the posterior view of the
liver-spleen scan is 13 cm [97]. It is considered enlarged if the posterior length is >14 cm, or if the lateral
scan area exceeds 80 cm2 [92]. In one study, 98 of 100 spleens with a posterior length 13 cm on colloid
scanning weighed less than 250 grams and were normal at postmortem examination [97].
CT scanning The spleen is enlarged if its length is >10 cm [98]. In one study this value had a
sensitivity, specificity, and accuracy of 81, 90, and 88 percent, respectively.
A study of spleen size by CT scanning was done in adults 17 to 88 years of age to determine if a single
measurement, as opposed to a volume determination, could be used to define splenomegaly [98]. The
authors evaluated splenic length, width, and thickness in 249 CT scans and assessed the spleen's
relationship to the left lobe of the liver and the inferior third of the left kidney. Based upon an upper limit of 314
cubic cm for the volume of a normal spleen, they found a maximum normal spleen length of 9.8 cm, similar to
be above-noted value of 10 cm [98]. If the spleen extended below the lower third of the kidney, this was also
evidence for splenomegaly.
Using the above criteria as "gold standards" for normal and abnormal splenic size, studies concerning the
ability to palpate a normal or enlarged spleen have concluded that not all palpable spleens are abnormal; this
is especially true in asthenic individuals. In one study, for example, no splenic pathology was clinically evident
in 18 of 21 patients with palpable splenomegaly (at least two observers) and a posterior splenic length 13 cm
on liver-spleen scan [97].
Nevertheless, a palpable spleen usually means the presence of significant splenomegaly. As a general rule,
a spleen has to be increased in size by at least 40 percent before it becomes palpable [99]. However, it has
been estimated that 20 percent of spleens with an estimated weight of more than 900 grams are not palpable
[93].
Splenic length in children In one study using ultrasound imaging, splenic lengths in hematologically
normal children were as follows (values as mean [cm] 1 SD) [100]:
5 to 6 years 8.4 0.9
7 to 8 years 8.5 1.0
9 to 10 years 8.6 1.1
11 to 12 years 9.7 1.0
13 to 14 years 10.1 1.2
15 to 16 years 10.1 1.0
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CAUSES OF SPLENOMEGALY
General conditions The causes of an enlarged spleen are multiple; most reflect the presence of hepatic
or hematologic disease, infection, or inflammation (table 2). Such causes include the following [73]:
Splenic engorgement due to sequestration of red blood cells, as in hereditary spherocytosis or other
congenital or acquired hemolytic anemias. A significantly enlarged spleen is routinely seen in normal
subjects serving as hematopoietic cell donors following treatment with granulocyte colony-stimulating
factor [96,101].
Extramedullary hematopoiesis in the spleen can arise due to expansion of hematopoietic tissue in
various congenital hemolytic anemias (eg, thalassemia), or as a response to marrow replacement by
fibrosis or tumor (eg, primary or secondary myelofibrosis). (See "Clinical manifestations and diagnosis of
the thalassemias", section on 'Splenomegaly' and "Clinical manifestations and diagnosis of primary
myelofibrosis", section on 'Clinical manifestations'.)
Other causes for splenic engorgement include splenic trauma with intracapsular hematoma formation,
sequestration crisis in sickle cell disease, and portal hypertension. (See "Surgical management of splenic
injury in the adult trauma patient" and "Chronic portal vein thrombosis in adults: Clinical manifestations,
diagnosis, and management", section on 'Clinical manifestations' and "Overview of the clinical
manifestations of sickle cell disease", section on 'Splenic sequestration crisis'.)
Chronic inflammation or infection, as in systemic lupus erythematosus, rheumatoid arthritis, infective
endocarditis, or chronic malaria. (See "Clinical manifestations and diagnosis of Felty's syndrome",
section on 'Splenomegaly'.)
Lipid deposition disorders such as Gaucher disease (see "Gaucher disease: Pathogenesis, clinical
manifestations, and diagnosis")
Congenital causes for splenomegaly include splenic hemangioma, hamartoma, or cysts.
Splenic invasion with granulomatous (eg, Mycobacteria) or malignant hematologic disease (eg,
lymphoma, myeloma).
Primary vascular neoplasms of the spleen, the most common being splenic angiosarcoma [102,103]
Splenic metastases Non-hematologic malignancies rarely metastasize to the spleen. The most
common primary sites include breast, lung, colorectal, ovarian carcinomas, and melanoma in cases of cancer
involving multiple visceral sites and colorectal and ovarian carcinomas in cases in which there is a solitary
splenic lesion [104,105].
Common causes The relative frequency of the different causes of splenomegaly was evaluated in a
series of 449 patients who were studied retrospectively and categorized; the most common cause in each
category is shown in parentheses [106]:
Liver disease 33 percent (cirrhosis)
Hematologic malignancy 27 percent (lymphoma)
Infection 23 percent (AIDS, endocarditis)
Congestion or inflammation 8 percent (congestive failure)
Primary splenic disease 4 percent (splenic vein thrombosis)
Other or unknown 5 percent
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Massively enlarged spleen A spleen enlarged such that its lower pole is within the pelvis, or which has
crossed the midline into the right lower or right upper abdominal quadrants is considered to be massively
enlarged. There are only a few conditions that cause this degree of splenic enlargement, each of which
discussed elsewhere on the appropriate topic reviews:
Chronic myeloid leukemia
Myelofibrosis, primary or secondary to polycythemia vera or essential thrombocytosis
Gaucher disease
Lymphoma, usually indolent, including hairy cell leukemia [107]
Kala-azar (visceral leishmaniasis)
Hyperreactive malarial splenomegaly syndrome, also called tropical splenomegaly syndrome [108]
Beta thalassemia major or severe beta thalassemia intermedia
AIDS with Mycobacterium avium complex [106]
EVALUATION OF THE PATIENT
Initial approach The history may provide valuable clues as to the cause of splenomegaly. As examples:
A patient with chronic alcoholism or hepatitis and ascites probably has splenomegaly secondary to
cirrhosis and portal hypertension. (See "Cirrhosis in adults: Etiologies, clinical manifestations, and
diagnosis".)
A young adult with fatigue, fever, sore throat, and splenomegaly is likely to have infectious
mononucleosis or other viral infection. (See "Infectious mononucleosis in adults and adolescents",
section on 'Classic IM'.)
An older adult complaining of post-bath pruritus with a ruddy complexion and splenomegaly is likely to
have polycythemia vera. (See "Clinical manifestations and diagnosis of polycythemia vera".)
In the patient with systemic complaints such as fever, night sweats, malaise, and/or weight loss, an enlarged
spleen may reflect activity of a systemic disease that may have already been diagnosed, such as AIDS,
systemic lupus erythematosus, rheumatoid arthritis, sarcoidosis, malaria, tuberculosis, viral infection
(infectious mononucleosis, cytomegalovirus, hepatitis), or hematologic disorders (chronic myeloid leukemia,
chronic lymphocytic leukemia, hairy cell leukemia). In such cases, the spleen may revert to normal size when
the underlying disease is brought under control with appropriate therapy.
Unexplained splenomegaly A problem often confronting the examining clinician is when the patient
presents with splenomegaly for which no prior diagnosed or evident condition can be considered responsible.
A reasonable and standard approach to such patients begins with an accurate history (including recent travel
information and a history of prior malignancy), physical examination, complete blood count with white blood
cell differential and platelet count and examination of the peripheral blood smear, liver function studies,
urinalysis, and chest x-ray. Testing for the presence of antibodies to HIV should be considered when no other
causes for splenomegaly are apparent. Abnormalities found at this time should help guide the physician to an
appropriate diagnosis. (See 'Additional approaches' below.)
Imaging studies A variety of imaging techniques are available for assessment of splenic lesions (eg,
splenic cysts, other space-occupying lesions), including CT scanning, magnetic resonance imaging,
ultrasound, Tc-99m sulfur colloid scintigraphy, and 18F-FDG PET. Although the age of the patient, clinical
symptomatology, and imaging characteristics might help the radiologist arrive at the correct diagnosis
[72,73,109-111], one study has concluded that PET scanning offered no additional information over that
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obtained using CT scanning alone, and that a history of prior malignancy was the only independent predictor
for a splenic lesion being malignant (odds ratio 6.3; 95% CI 2.3-17) [105].
CBC and peripheral smear The complete blood count, examination of the peripheral smear, and white
blood cell differential are often of utmost importance in determining the cause of an enlarged spleen:
Neutropenia, anemia, and/or thrombocytopenia may be present, as these formed elements can be
trapped in an enlarged spleen, giving the nonspecific picture termed "hypersplenism." (See "Extrinsic
nonimmune hemolytic anemia due to mechanical damage: Fragmentation hemolysis and
hypersplenism", section on 'Extravascular nonimmune hemolysis due to hypersplenism'.)
There is no predictable relationship between the degree of splenomegaly and the presence or degree of
these cytopenias or of possible associated symptoms such as colicky abdominal or left upper quadrant
pain, pain while lying on one's side, or early satiety [4].
The cytopenias seen with hypersplenism are not always associated with abnormal white blood cell or red
blood cell forms. However, cytopenias are nonspecific, as multiple other causes, including certain
infections, can provoke these changes in a patient with splenomegaly.
Neutrophilia (ie, absolute neutrophil count >7700/microL) with or without increased numbers of band and
metamyelocyte forms ("left shift"), suggest the presence of infection. (See "Approach to the patient with
neutrophilia".)
On occasion, invading organisms may be seen on the peripheral smear, either free in the plasma as in
overwhelming sepsis, or within neutrophils or monocytes (bacteria, ehrlichiae) or red blood cells as
occurs with bartonellosis, babesiosis (picture 3), and malaria (picture 4A-B). (See "Extrinsic nonimmune
hemolytic anemia due to systemic disease", section on 'Direct parasitization'.)
Patients with overwhelming bacterial sepsis may show other abnormalities on smear, including
microangiopathic changes in red blood cells (picture 5) and toxic granulation, vacuoles, and Dohle bodies
in neutrophils (picture 6). (See "Approach to the patient with neutrophilia", section on 'Evaluation of the
complete blood count'.)
Certain infectious organisms are associated with relatively specific changes, such as red blood cell
agglutination due to the presence of cold agglutinins (picture 7) in infections with Mycoplasma
pneumoniae or in infectious mononucleosis, and the atypical lymphocytes seen in the latter infection
(picture 8). (See "Pathogenesis of autoimmune hemolytic anemia: Cold agglutinin disease" and
"Infectious mononucleosis in adults and adolescents".)
The combination of sepsis and profound anemia along with hemoglobinemia, hemoglobinuria, and the
presence of microspherocytes and red cell ghosts on the peripheral blood smear (ie, massive
intravascular hemolysis) suggests infection with a phospholipase-producing Clostridial organism. (See
"Clostridial myonecrosis", section on 'Clinical manifestations' and "Extrinsic nonimmune hemolytic
anemia due to systemic disease", section on 'Clostridium perfringens'.)
The presence of white blood cells less mature than band forms and nucleated red cells on the peripheral
smear, along with teardrop-shaped red cells (ie, a leukoerythroblastic blood picture) (picture 9), suggests
the presence of widespread bone marrow invasion with malignancy. (See "Anemias due to decreased
red cell production", section on 'Leukoerythroblastic anemia'.)
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This picture may also be seen in tuberculosis and chronic myeloid leukemia and is quite typical for
primary and secondary forms of myelofibrosis. These three diagnoses can be established through culture
and staining techniques, the presence of the bcr-abl construct or Ph1 chromosome, and bone marrow
biopsy, respectively.
Patients with neutropenia and rheumatoid arthritis may have an increased percentage of circulating large
granular lymphocytes (picture 10). (See "Large granular lymphocyte leukemia in rheumatoid arthritis".)
The presence of increased numbers of abnormal cells in the peripheral blood suggests the presence of a
hematologic malignancy. Morphologic evaluation, coupled with special staining and immunocytochemical
techniques, can help to establish the correct diagnosis [112]. (See "Evaluation of the peripheral blood
smear", section on 'Worrisome findings'.)
Additional approaches
General considerations If no abnormalities are suggested by the above workup, CT examination of
the chest and abdomen should be performed to evaluate the patient for disseminated or intra-abdominal
malignancy, such as hepatoma or lymphoma, advanced liver disease, or portal hypertension. Consultation
with a hematologist would be appropriate at this time, especially if examination of the peripheral blood smear
has not been performed, or if changes are present which cannot be adequately assessed by the patient's
primary clinician.
An alternative approach is to biopsy tissue depending upon the clinical suspicion. Thus, if infection is
suspected, a lymph node or bone marrow aspiration and biopsy may be indicated, a liver biopsy if liver
disease is suspected, and a bone marrow biopsy if a hematologic disorder is suspected [106]. Without a
specific organ or tissue to biopsy, a reasonable approach would be performance of a bone marrow aspiration
with biopsy with culture. Conditions such as lipid storage diseases, disseminated mycobacterial or
granulomatous disease, and lymphoid or myeloid disorders may be diagnosed in this way (picture 11 and
picture 12). (See "Gaucher disease: Pathogenesis, clinical manifestations, and diagnosis".)
Diagnostic splenectomy If the above procedures have not led to a diagnosis (eg, abdominal
sonography, whole body CT examination, bone marrow aspiration and biopsy, examination of the peripheral
smear, tests of liver and kidney function [113]), splenectomy may be considered, with the value of making a
diagnosis balanced by considerations not only of surgical morbidity and mortality, but also of disease-specific
complications of splenectomy [2]. (See "Hereditary spherocytosis: Clinical features, diagnosis, and treatment",
section on 'Splenectomy' and "Treatment of beta thalassemia", section on 'Splenectomy' and "Management of
primary myelofibrosis", section on 'Splenectomy' and "Sideroblastic anemias: Diagnosis and management",
section on 'Avoidance of splenectomy'.)
The resected spleen may reveal a localized splenic tumor, cyst(s), inflammatory pseudotumor, hamartoma,
amyloid, vascular anomaly, infection, sarcoidosis, or other rare condition [114,115]. In some cases, a
satisfactory answer may not be available even after pathologic examination. In a series of 122 "diagnostic"
splenectomies performed for unexplained splenomegaly, splenic mass lesion, or to accurately classify a
lymphoproliferative disorder detected but not further characterizable on bone marrow or peripheral blood
examination, the most common pathologic diagnoses were [116]:
Lymphoma/leukemia 57 percent
Metastatic carcinoma/sarcoma 11 percent
Cyst/pseudocyst 9 percent
Benign/malignant vascular neoplasm 7 percent
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In the subgroup of 41 patients undergoing splenectomy for splenomegaly without a splenic mass lesion or
evidence of a lymphoproliferative disorder, lymphoma/leukemia was still the most common pathologic
diagnosis (58 percent). Of interest, no distinct histologic abnormality was found in 5 percent of the 122
resected spleens. Similar results were reported in two series of patients undergoing diagnostic splenectomy,
in which a lymphoma was found in 37 and 39 percent of the resected spleens [117,118].
Splenic aspiration/biopsy Splenic aspiration/biopsy is not widely practiced in the United States
because of a concern for bleeding. As an example, a 1985 study reported a major complication rate of 13
percent for percutaneous biopsy of the spleen performed with a 14 gauge needle [119]. However, subsequent
reports of fine needle aspiration/biopsy of the spleen under radiologic or ultrasound guidance have indicated
much lower complication rates using smaller diameter needles (ie, 18 gauge or smaller), allowing for a safer
procedure along with avoidance of the need for major surgical intervention (ie, open splenic biopsy or
splenectomy) [120-124]. Another series of 92 patients who underwent 97 core needle biopsies of the spleen
at a single institution found minor complications (pain, asymptomatic bleeding) associated with seven
procedures (7.2 percent) and a major complication (hemothorax requiring chest tube placement and blood
transfusion) associated with one procedure [125].
In a meta-analysis that included four studies meeting inclusion criteria for diagnostic accuracy (639 patients)
and nine studies meeting inclusion criteria for complication rates (741 patients), it was concluded that imageguided percutaneous biopsy of the spleen demonstrated high diagnostic accuracy, with a sensitivity and
specificity of 87 and 96 percent, respectively [126]. The pooled overall complication rate was 4.2 percent
(core needle biopsy: 5.8 percent, fine needle aspiration biopsy: 4.3 percent). However, sensitivity analysis
with the removal of biopsies performed with needles larger than 18 gauge showed overall and major
complication rates of 3.9 and 1.3 percent, respectively, similar to those reported for biopsies of the liver and
kidney.
While splenic aspiration/biopsy is safer than it was in the past, we and others believe that this procedure is of
minimal clinical utility, does not provide information that could not be obtained by other means, and is not
useful in preventing an operation [105].
SPLENECTOMY
Morbidity and mortality of the procedure Despite its important functions, the spleen can be removed, in
whole or in part, for therapeutic or diagnostic purposes, either via classical open laparotomy or laparoscopic
approaches. Its position in the left upper abdominal quadrant places it in close proximity to a number of
important structures (figure 1), including the diaphragm, stomach (figure 4), tail of the pancreas (figure 2), left
kidney, and the splenic flexure of the large intestine (figure 5). Accordingly, removal of the spleen may place
one or more of these structures and/or their vascular supplies at risk.
While postoperative outcomes are generally more favorable with laparoscopic approaches [127,128], either
can be associated with serious complications (eg, risk of infection with encapsulated organisms [especially in
the pediatric population], pneumonia, intra- and postoperative hemorrhage, thrombocytosis with or without
venous thromboembolism, pancreatitis, gastric fistula) [129], with reported morbidities and mortalities in
patients with hematologic disorders ranging from 8 to 52 percent and zero to 9 percent, respectively
[130-137]. (See "Surgical management of splenic injury in the adult trauma patient", section on 'Surgical
outcomes and complications'.)
As an example, in a report of 381 patients who underwent splenectomy for hematologic disease between
1997 and 2010, the following results were reported [130]:
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Open or laparoscopic splenectomy was performed in 75.6 and 21.8 percent of the patients, respectively.
A laparoscopic operation was converted to open surgery in 2.6 percent.
The median durations of open and laparoscopic operations were 90 (range: 26 to 500) and 119 (range:
25 to 372) minutes, respectively, with estimated blood losses of 250 (range: 5 to 13,000) and 150 (range:
5 to 2,000) mL, respectively.
Transfusion of whole blood or platelets was required in 23 and 24 percent of the procedures,
respectively.
The median in hospital lengths of stay were six (range: 1 to 203) and three (range: 1 to 18) days for the
open and laparoscopic procedures, respectively.
Morbidity was 40 and 17 percent for the open and laparoscopic procedures, respectively. Although most
complications were minor, 42 percent were related to infection. On multivariate analysis, independent
predictors of major complications were age >65, platelet count 50,000/microL, and a Karnofsky
performance score 60 (table 3).
Twenty-four (6.3 percent) of the 381 patients died within 30 days of surgery; 75 percent of the deaths
were directly related to an infectious complication.
Long-term risks A number of prior studies have evaluated both the short- and long-term risks attendant to
splenectomy. While these have generally concluded that there is an increased risk for both infection and
thrombosis, the influence of splenectomy on the subsequent incidence of malignancy is unclear [138-142].
(See "Surgical management of splenic injury in the adult trauma patient", section on 'Surgical outcomes and
complications' and "Clinical features and management of sepsis in the asplenic patient" and "Treatment of
beta thalassemia", section on 'Splenectomy' and "Hereditary spherocytosis: Clinical features, diagnosis, and
treatment", section on 'Splenectomy'.)
To understand long-term risks and complications of this procedure, a follow-up study was undertaken in 8149
cancer-free subjects in the Veterans Administration (VA) database who had undergone splenectomy, with a
mean follow-up of 12.6 years. Results included the following, with all comparisons being to a random sample
of 6731 non-splenectomized VA patients matched on the basis and race and year of birth [143]:
Infection Splenectomized patients had a significantly increased risk of pneumococcal pneumonia (RR
2.1), pneumonia not otherwise specified (RR 1.9), meningitis (RR 2.4), and septicemia (RR 3.4), as well
as a significantly increased risk of death from pneumonia (RR 1.6) and septicemia (RR 3.0). These risks
were independent of patient age, presence of autoimmune disease, or trauma. The risks of infection and
death from infection were significantly elevated more than 10 years following splenectomy.
Thromboembolism Splenectomized patients had a significantly increased risk of developing deep vein
thrombosis and pulmonary embolism (RR 2.2 for both), but not acute myocardial infarction, coronary
artery disease, or ischemic stroke. Risks did not differ when analyzing for race, age at splenectomy,
trauma, or prior autoimmune disease.
Malignancy Malignancy developed in 13 percent of the splenectomized patients during follow-up.
There was a significantly increased risk of any cancer as well as death from any cancer (RR 1.5 for both).
Subgroup analysis revealed an increased risk of death from cancer of the liver (RR 1.8), pancreas (RR
2.2), lung (RR 1.3), non-Hodgkin lymphoma (RR 4.7), and any leukemia (RR 2.4). Risks were similar
when stratified by race, age at splenectomy, and trauma. Risks for most of the malignancies tended to be
highest during the first two to five years post-splenectomy. However, after more than 10 years, there was
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still a significantly increased risk of esophageal, liver, and lung cancers, non-Hodgkin lymphoma, Hodgkin
lymphoma, acute myeloid leukemia, chronic lymphocytic leukemia, chronic myeloid leukemia, and any
leukemia.
Risks of malignancy were also evaluated in a population-based cohort study in Taiwan that followed 2295
patients with traumatic splenectomy, 2603 patients with non-traumatic splenectomy, and 19,592 age-matched
controls [144]. Compared with controls, patients who underwent splenectomy had a higher incidence of
malignancy (9.73 versus 5.03 per 1000 person-years; adjusted hazard ratio [HR] 2.06; 95% CI 1.81-2.33).
The risk was relatively unaffected by the indication for splenectomy, and a variety of cancer types were
involved, including non-Hodgkin lymphoma, leukemia, and upper gastrointestinal and hepatic malignancies.
When to consider splenectomy With the exception of isolated splenic lesions, there is no condition for
which splenectomy alone is curative. Given the morbidity and mortality of the procedure, clinical judgement is
required when deciding whether to perform this procedure in a particular patient. The following four clinical
examples indicate the complexity of this issue:
Splenic trauma Removal of the spleen versus salvage procedures. (See "Surgical management of
splenic injury in the adult trauma patient", section on 'Splenectomy versus salvage'.)
Second line treatment in immune thrombocytopenia Splenectomy versus other second line agents
(eg, rituximab, thrombopoietin receptor agonists) when first line agents (ie, glucocorticoids) have failed.
(See "Immune thrombocytopenia (ITP) in adults: Second-line and subsequent therapies".)
Splenomegaly in primary myelofibrosis Splenectomy versus other therapeutic modalities (eg,
splenic irradiation, hydroxyurea, ruxolitinib) for the patient with symptoms secondary to an enlarged
spleen. (See "Management of primary myelofibrosis", section on 'Splenectomy'.)
Hereditary spherocytosis Total or partial splenectomy versus supportive care, with considerations
including surgical approach, need for cholecystectomy for concomitant bilirubin gallstones, heightened
risk for postoperative sepsis, arterial and venous thromboembolism, as well as the optimal age for the
procedure. (See "Hereditary spherocytosis: Clinical features, diagnosis, and treatment", section on
'Splenectomy'.)
General indications for splenectomy include the following:
Isolated thrombocytopenia, hemolytic anemia, or neutropenia
Painfully enlarged spleen
Traumatic or atraumatic splenic rupture (see 'Splenic rupture' above)
Splenic artery aneurysm (see 'Splenic artery aneurysm' above)
To allow adjuvant treatment when multiple cytopenias due to hypersplenism are present
Primary treatment of an isolated splenic vascular or parenchymal lesion
To allow diagnosis in the case of splenomegaly of unclear cause (see 'Additional approaches' above)
Specific conditions in which splenectomy may be considered There are many conditions for which
splenectomy might be considered. For each of the following, the relevant UpToDate topic review should be
consulted for specific indications, therapeutic alternatives, complications, and expected results.
Immune thrombocytopenia (ITP)
Autoimmune hemolytic anemia, warm type (AIHA)
Thalassemia major or intermedia
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Hereditary spherocytosis
Primary myelofibrosis
Hairy cell leukemia, splenic marginal zone lymphoma, chronic lymphocytic leukemia
Splenic sequestration crisis in sickle cell disease
Acute splenic torsion with infarction due (eg, "wandering spleen syndrome")
Blunt abdominal trauma with splenic contusion or rupture
Splenic abscess or infarction
Splenic vein thrombosis with bleeding esophageal varices
Splenorenal shunting for portal hypertension
Feltys syndrome
HLA/ABO desensitization for renal transplantation
Combined with cytoreductive therapy for advanced ovarian carcinoma
As an aid during surgical removal of regional tumors (eg, pancreas, stomach, kidney)
SUMMARY AND RECOMMENDATIONS
The spleen participates in cellular and humoral immunity, removes particulates, bacteria, and senescent
or poorly deformable red blood cells from the circulation, and contains approximately one-third of
circulating platelets. Under abnormal circumstances the spleen may become the site of extramedullary
hematopoiesis. (See 'Normal splenic function' above.)
The median splenic weight in adults is about 150 grams and the organ is not usually palpable on physical
examination. On ultrasound the spleen is considered to be normal in size if its length is <13 cm. (See
'Splenic size and palpability' above.)
Symptoms of an enlarged or abnormal spleen include pain, fullness, or discomfort in the left upper
abdominal quadrant or referred to the left shoulder. A pleuritic component and/or early satiety may also
be present. (See 'Symptoms' above.)
Splenic abnormalities can include hypersplenism, hyposplenism, asplenia, abscess, infarction,
calcification, cysts, and splenic rupture. (See 'Splenic disorders' above.)
The major causes for an enlarged spleen are shown in the table (table 2). Only a few disorders are
associated with a massively enlarged spleen (ie, lower pole within the pelvis or the splenic edge has
crossed the midline). (See 'Causes of splenomegaly' above and 'Massively enlarged spleen' above.)
The patient evaluation and role of splenectomy are discussed above. (See 'Evaluation of the patient'
above and 'Splenectomy' above.)
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Topic 7134 Version 51.0
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GRAPHICS
Anterior view of upper abdominal structures
The spleen is located in the left upper quandrant of the abdomen beneath the
left hemidiaphragm and lateral to the greater curvature of the stomach.
Reproduced with permission from: Bickley, LS, Szilagyl, P. Bates' Guide to Physical
Examination and History Taking, 8th Ed. Philadelphia: Lippincott Williams & Wilkins
2003. Copyright 2003 Lippincott Williams & Wilkins.
Graphic 62682 Version 2.0
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Vascular supply of the spleen
The splenic artery is a branch of the celiac trunk. It traverses along the superior margin of the
pancreas toward the spleen branching to form up to six major arteries before entering the
spleen. The splenic vein joins the superior mesenteric vein to form the portal vein.
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Effect of spleen size on platelet pooling
This figure shows the two-hour recovery in the general circulation of

radioactively-labeled platelets transfused to asplenic (red), normal (green), and
splenomegalic (orange) patients. The vast majority of the splenomegalic
patients had congestive splenomegaly secondary to cirrhosis with portal
hypertension.
Adapted from: Aster RH. Pooling of platelets in the spleen: role in the pathogenesis of
'hypersplenic' thrombocytopenia. J Clin Invest 1966; 45:645.
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Major causes of reactive thrombocytosis

Nonmalignant hematologic conditions
Acute blood loss
Acute hemolytic anemia
Iron deficiency anemia
Treatment of vitamin B12 deficiency
Rebound effect after treatment of immune thrombocytopenia (ITP)
Rebound effect after ethanol-induced thrombocytopenia
Malignant conditions
Metastatic cancer
Lymphoma
Rebound effect following use of myelosuppressive agents
Acute and chronic inflammatory conditions

Rheumatologic disorders, vasculitides
Inflammatory bowel disease
Celiac disease
Kawasaki disease
Nephrotic syndrome
POEMS syndrome (osteosclerotic myeloma)
Tissue damage
Thermal burns
Myocardial infarction
Severe trauma
Acute pancreatitis
Post-surgical period, especially post-splenectomy
Coronary artery bypass procedures
Infections
Chronic infections
Tuberculosis
Acute bacterial and viral infections
Exercise
Allergic reactions
Functional and surgical asplenia
Reaction to medications
Vincristine
Epinephrine, glucocorticoids
Interleukin-1B
All-trans retinoic acid
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Thrombopoietin, thrombopoietin mimetics

Low molecular weight heparins (enoxaparin)
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Howell-Jolly bodies following splenectomy
This peripheral blood smear shows two red blood cells (RBCs) that contain
Howell-Jolly bodies (black arrows). Howell-Jolly bodies are remnants of RBC
nuclei that are normally removed by the spleen. Thus, they are seen in patients
who have undergone splenectomy (as in this case) or who have functional
asplenia (eg, from sickle cell disease). Target cells (blue arrows) are another
consequence of splenectomy.
Courtesy of Carola von Kapff, SH (ASCP).
Normal peripheral blood smear
High-power view of a normal peripheral blood smear. Several platelets

(arrows) and a normal lymphocyte (arrowhead) can also be seen. The red
cells are of relatively uniform size and shape. The diameter of the normal red
cell should approximate that of the nucleus of the small lymphocyte; central
pallor (dashed arrow) should equal one-third of its diameter.
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Peripheral blood smear in sickle cell anemia
Peripheral blood smear from a patient with sickle cell anemia. This smear shows
multiple sickle cells (blue arrows). There are also findings consistent with
functional asplenia, including a nucleated red blood cell (upper left), a red blood
cell containing a Howell-Jolly body (black arrow), and target cells (red arrow).

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Splenosis on CT scan
This 55 year-old woman had a total splenectomy for traumatic splenic rupture. A CT scan taken a
number of years later for unrelated purposes showed two foci of splenosis (arrows) and a surgical clip
(arrowhead).
CT: computed tomography.
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Splenosis causing small bowel obstruction on CT scan
A CT scan reformatted in the coronal plane (A) shows an empty splenic bed (short arrow), multiple
peritoneal nodules (arrowheads), and dilated small bowel (dashed arrow). An axial image through the
abdomen (B) shows multiple splenic peritoneal implants (arrowheads), dilated upstream small bowel
(dashed arrow), and decompressed distal small bowel (arrow).
CT: computed tomography; SBO: small bowel obstruction.
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Calcified splenic artery aneurysms on CT
An axial CT scan through the upper abdomen (left image) shows two splenic artery aneurysms with calcified walls (arrows). The
image on the right from the same patient shows a third 4 cm aneurysm (asterisk) with a calcified wall (arrow). The aneurysms
were an incidental finding.
CT: computed tomography.
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Major causes of splenomegaly

Congestive
Cirrhosis
Heart failure
Thrombosis of portal, hepatic, or splenic veins
Malignancy
Lymphoma, usually indolent variants
Acute and chronic leukemias
Polycythemia vera
Multiple myeloma and its variants
Essential thrombocythemia
Primary myelofibrosis
Primary splenic tumors
Metastatic solid tumors
Infection
Viral - hepatitis, infectious mononucleosis, cytomegalovirus
Bacterial - salmonella, brucella, tuberculosis
Parasitic - malaria, schistosomiasis,toxoplasmosis, leishmaniasis
Infective endocarditis
Fungal
Inflammation
Sarcoid
Serum sickness
Systemic lupus erythematosus
Rheumatoid arthritis (Felty syndrome)
Infiltrative, nonmalignant
Gaucher's disease
Niemann-Pick disease
Amyloid
Glycogen storage disease
Langerhans cell histiocytosis
Hemophagocytic lymphohistiocytosis
Rosai-Dorfman disease
Hematologic (hypersplenic) states

Acute and chronic hemolytic anemias, all etiologies
Sickle cell disease (children)
Following use of recombinant human granulocyte colony-stimulating factor
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Babesia microti parasites in red blood cells

Image
Panel A) Babesia microti [1]. This thin peripheral blood smear (Giemsa stain; x1000)
shows Babesia microti. Several erythrocytes contain multiple parasites, including a
diagnostic tetrad form (arrow). Panel B) Malaria (for comparison) [2]. Peripheral smear
from a patient with malaria shows intraerythrocytic ring forms (trophozoites) (arrows).
Panel C) Normal [2]. High power view of a normal peripheral blood smear. Several
platelets (black arrows) and a normal lymphocyte (blue arrow) can also be seen. The red
cells are of relatively uniform size and shape. The diameter of the normal red cell should
approximate that of the nucleus of the small lymphocyte; central pallor (red arrow)
should equal one-third of its diameter.
Courtesy of:
1. Harriet Provine
2. Carola von Kapff, SH (ASCP).
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Plasmodium vivax
Giemsa-stained thin smear of blood (x1000) shows a ring trophozoite of

Plasmodium vivax. Red blood cells infected by P. vivax are enlarged and contain
Schffner's dots.
Courtesy of Harriet Provine.
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Blood smear of falciparum malaria
Giemsa- stained thin smear of blood showing the characteristic banana-shaped

gametocyte of Plasmodium falciparum (arrow).
Courtesy of Stephen B Calderwood, MD.
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Peripheral smear in microangiopathic hemolytic anemia

showing presence of schistocytes
Peripheral blood smear from a patient with a microangiopathic hemolytic anemia

with marked red cell fragmentation. The smear shows multiple helmet cells
(arrows) and other fragmented red cells (small arrowhead); microspherocytes
are also seen (large arrowheads). The platelet number is reduced; the large
platelet in the center (dashed arrow) suggests that the thrombocytopenia is due
to enhanced destruction.

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Toxic granulations and Dhle bodies in infection/inflammation

Image
Left panel: Peripheral blood smear shows neutrophils with toxic granulations, which are
dark coarse granules. A Dhle body is also seen (arrow). Right panel: A neutrophil with
toxic granulations, vacuoles (another toxic change), and a Dhle body (arrow). These
abnormalities are characteristic of toxic systemic illnesses.

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Red blood cell agglutination due to a cold agglutinin
Peripheral blood smear from a patient with cold agglutinin hemolytic anemia
shows marked red blood cell agglutination into irregular clumps.

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Atypical lymphocytes in infectious mononucleosis
Peripheral smear from a patient with infectious mononucleosis shows three

atypical lymphocytes with generous cytoplasm.
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Leukoerythroblastic peripheral blood smear

Image
Leukoerythroblastic peripheral blood smear showing the presence of nucleated

red cells and immature white cells. This pattern occurs with marrow
replacement, usually due to fibrosis that may be idiopathic (eg, primary
myelofibrosis) or reactive to conditions such as metastatic cancer.

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Small lymphocytes versus large granular lymphocytes
Distinction between small lymphocytes (A) and large granular lymphocytes (B)
on blood smears. The large granular lymphocyte is about twice the size of the
red cells and has abundant cytoplasm containing azurophilic granules.
Micrographs were viewed with a Leica Leitz DMRB microscope using a 100x/1.30
oil immersion objective. Images were captured with a Sony Exwave HAD camera
and manipulated using Tribyn Version 1.3 software.
This research was originally published in Blood. Lamy T, Loughran TP Jr. How I treat
LGL leukemia. Blood 2011; 117:2764. Copyright 2011 American Society of
Hematology.
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Niemann Pick Cells on bone marrow aspirate

Image
Bone marrow aspirate from a patient with Niemann Pick disease, showing two
large macrophages laden with sphingomyelin, giving the cytoplasm a "foamy"
appearance (arrows).
Courtesy of David S Rosenthal, MD and William C Moloney, MD.
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Gaucher cells on bone marrow aspirate

Image
Bone marrow aspirate showing a number of large macrophages laden with

cerebrosides (Gaucher cells, arrows) in a patient with Gaucher disease and
concomitant multiple myeloma. The cytoplasm has a pattern which has been
likened to wrinkled silk or crumpled newspaper.
Courtesy of David S Rosenthal, MD and William C Moloney, MD.
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Anatomy of the stomach

Image
The relationship of the stomach to surrounding structures is depicted in the figure. The arterial
supply to the stomach is derived primarily from the celiac axis. The celiac axis arises from the
proximal abdominal aorta and typically branches into the common hepatic, splenic, and left
gastric arteries. The common hepatic artery usually gives rise to the gastroduodenal artery (in
approximately 75 percent of people), which, in turn, branches off into the right gastroepiploic
artery and the anterior and posterior superior pancreaticoduodenal arteries, which supply the
pancreas. The right gastroepiploic artery joins with the left gastroepiploic artery, which
emanates from the splenic artery in 90 percent of patients. The right gastric artery branches
from the hepatic artery and anastomoses with the left gastric artery along the lesser curvature
of the stomach. Because of its highly redundant blood supply, stomach ischemia is rare.
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Anatomic relationship of colon to surrounding structures

Image
This figure depicts the relationship of the large intestine to the overlying and underlying
organs and vessels.
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Karnofsky Performance Status scale

Value
100
90
Level of functional capacity
Definition
Normal, no complaints, no evidence of
Able to carry on normal activity and to work; no special
disease
care needed
Able to carry on normal activity, minor signs

or symptoms of disease
80
Normal activity with effort, some signs or

symptoms of disease
70
60
Cares for self, unable to carry on normal
Unable to work; able to live at home and care for most
activity or to do active work
personal needs; various degrees of assistance needed
Requires occasional assistance but is able to

care for most needs
50
Requires considerable assistance and

frequent medical care
40
30
Disabled, requires special care and
Unable to care for self; requires equivalent of institutional
assistance
or hospital care; disease may be progressing rapidly
Severely disabled, hospitalization is

indicated although death is not imminent
20
Hospitalization is necessary, very sick,

active supportive treatment necessary
10
Moribund, fatal processes progressing

rapidly
Dead
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Contributor Disclosures
Stanley L Schrier, MD Nothing to disclose William C Mentzer, MD Equity Ownership/Stock Options:
Johnson & Johnson [Anemia (Erythropoietin)]. Jennifer S Tirnauer, MD Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform
to UpToDate standards of evidence.
Conflict of interest policy
12/25/2016 12:43 PM

Approach To The Adult Patient With Splenomegaly and Other Splenic Disorders

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or young adults [9,10].

abscess or rupture may require surgical intervention.

(See "Initial evaluation and management of blunt abdominal trauma in adults".)

measurements in centimeters [94].

with sickle cell disease. Exp Hematol 2007; 35:179.

Vascular supply of the spleen

Effect of spleen size on platelet pooling

This figure shows the two-hour recovery in the general circulation of

Major causes of reactive thrombocytosis

Acute and chronic inflammatory conditions

Thrombopoietin, thrombopoietin mimetics

Howell-Jolly bodies following splenectomy

Normal peripheral blood smear

High-power view of a normal peripheral blood smear. Several platelets

Peripheral blood smear in sickle cell anemia

Normal peripheral blood smear

High-power view of a normal peripheral blood smear. Several platelets

Splenosis causing small bowel obstruction on CT scan

Calcified splenic artery aneurysms on CT

Major causes of splenomegaly

Hematologic (hypersplenic) states

Babesia microti parasites in red blood cells

Giemsa-stained thin smear of blood (x1000) shows a ring trophozoite of

Blood smear of falciparum malaria

Giemsa- stained thin smear of blood showing the characteristic banana-shaped

Peripheral smear in microangiopathic hemolytic anemia

Peripheral blood smear from a patient with a microangiopathic hemolytic anemia

Normal peripheral blood smear

High-power view of a normal peripheral blood smear. Several platelets

Toxic granulations and Dhle bodies in infection/inflammation

Normal peripheral blood smear

High-power view of a normal peripheral blood smear. Several platelets

Graphic 59683 Version 4.0

Red blood cell agglutination due to a cold agglutinin

Normal peripheral blood smear

High-power view of a normal peripheral blood smear. Several platelets

Atypical lymphocytes in infectious mononucleosis

Peripheral smear from a patient with infectious mononucleosis shows three

Leukoerythroblastic peripheral blood smear

Leukoerythroblastic peripheral blood smear showing the presence of nucleated

Normal peripheral blood smear

High-power view of a normal peripheral blood smear. Several platelets

Small lymphocytes versus large granular lymphocytes

Niemann Pick Cells on bone marrow aspirate

Gaucher cells on bone marrow aspirate

Bone marrow aspirate showing a number of large macrophages laden with

Anatomy of the stomach

Anatomic relationship of colon to surrounding structures

Karnofsky Performance Status scale

Level of functional capacity

Normal, no complaints, no evidence of

Able to carry on normal activity and to work; no special

Able to carry on normal activity, minor signs

Normal activity with effort, some signs or

Cares for self, unable to carry on normal

Unable to work; able to live at home and care for most

activity or to do active work

personal needs; various degrees of assistance needed

Requires occasional assistance but is able to

Requires considerable assistance and

Disabled, requires special care and