DIAGNOSTIC DILEMMA

Aimee K. Zaas, MD
Thomas J. Marrie, MD, Section Editors

A Diagnostic Pitfall: Salmonella Splenic

Infarction in Hereditary Spherocytosis
Siddharth Sridhar, MRCP,a Susanna K.P. Lau, MD,a,b,c Patrick C.Y. Woo, MDa,b,c
a

Department of Microbiology, bState Key Laboratory of Emerging Infectious Diseases, cResearch Centre of Infection and Immunology,
The University of Hong Kong, Hong Kong SAR, China.

PRESENTATION
An initial working diagnosis of community-acquired pneumonia was ultimately superseded by a very different
conclusion. The patient, a 21-year-old male university student, had a 10-day history of fever, cough, and left-sided
pleuritic chest pain. He had hereditary spherocytosis but
was transfusion independent and had not undergone splenectomy. A 7-day course of amoxicillin-clavulanate before
admission provided no improvement.

ASSESSMENT
Physical examination showed pallor, percussion dullness, and
diminished breath sounds over the left chest. An abdominal
examination revealed cholecystectomy scars and splenomegaly. The patients hemoglobin level was 60 g/L and a total
leukocyte count was 14.94  103 cells/mcL. His chest radiograph disclosed a left pleural effusion (Figure 1). Diagnostic
pleurocentesis demonstrated purulent pleural uid with a
markedly elevated total cell count (4.092  103 cells/mcL)
that was neutrophil predominant. The pleural uid/serum
lactate dehydrogenase ratio was 0.8, substantiating the
presence of an exudative effusion by Lights criteria. A
diagnosis of community-acquired pneumonia complicated by
parapneumonic effusion was made, and broad-spectrum antimicrobial coverage with piperacillin-tazobactam and azithromycin was prescribed.
Bacterial cultures of pleural uid and blood were negative. Streptococcus pneumoniae and Legionella pneumophila antigens were not detectable in urine. Pleural uid
Funding: None.
Conict of interest: None.
Authorship: All authors had access to the data and had a role in writing
the manuscript.
Requests for reprints should be addressed to Patrick C.Y. Woo, MD,
Department of Microbiology, The University of Hong Kong, University
Pathology Building, Queen Mary Hospital, 102 Pokfulam Road, Hong
Kong.
E-mail address: pcywoo@hku.hk
0002-9343/$ -see front matter 2016 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.amjmed.2015.07.024

tests for Mycobacterium tuberculosis and Mycoplasma

pneumoniae by polymerase chain reaction were also
negative.
However, the patients fever persisted despite antibiotics.
In view of his poor clinical response, computerized tomography (CT) was ordered to assess his lungs and upper
abdomen. Imaging demonstrated a large infarction in the
superior pole of the spleen (Figure 2). Compressive
atelectasis was noted in the lower zone of the left lung,
but there was no evidence of consolidation. Laparotomy
revealed a necrotic splenic pole with foul-smelling pus,
and the spleen was removed. Culture of the pus and spleen
tissue yielded Gram-negative bacteria that were identied to
be Salmonella enteritidis.

Figure 1 A chest radiograph obtained on admission showed

left pleural effusion.

Sridhar et al

Massive Splenic Infarction due to Non-typhoidal Salmonellosis in Hereditary Spherocytosis

43

gastroenteritis and had received antibiotics prior to admission that could have lowered the sensitivity of blood cultures for Salmonella. Furthermore, he presented with a left
pleural effusion, which occasionally coincides with splenic
pathology, rather than the typical acute spleen syndrome of
left upper-quadrant abdominal pain radiating to the shoulder.6 Together, these factors compounded the diagnostic
uncertainty as to the cause of the pleural effusion, delaying
denitive surgical drainage to control the infection.

MANAGEMENT

Figure 2 Abdominal contrast-enhanced computed tomography demonstrated a large splenic infarct.

DIAGNOSIS
The patients diagnosis was splenic infarction due to invasive nontyphoidal salmonellosis. Splenic infarcts are an
important diagnostic pitfall in patients presenting with leftsided pleural effusions and no underlying lung pathology.
This is particularly true in patients with hematological disorders, such as hypercoagulable states, myeloproliferative
neoplasms, and sickle cell disease. It is essential to note that
patients with hemoglobinopathy have heterogeneous risk for
splenic infarction.
Although repeated splenic infarctions due to intrasplenic
sickling is a feature of sickle cell disease, such infarctions are
seldom observed in other forms of hemoglobinopathy, such
as thalassemia major or hereditary spherocytosis.1,2 Even in
adults with sickle cell disease, spontaneous massive splenic
infarction is rare without acute environmental triggers such as
altitude exposure and physical stress.3 Therefore, when a
patient with a hemoglobinopathy has a large splenic infarction without an environmental precipitant, a rigorous investigation for infection should follow.
The strong association between hemoglobinopathy and
invasive nontyphoidal salmonellosis dictates that Salmonella
be considered in choosing empirical therapy before conclusive cultures are available. An explanation for this relationship, once it is identied, is likely to be multifactorial.
Neutrophil dysfunction, demonstrated in patients with sickle
cell disease and in those with thalassemia, is a possible
consequence of intracellular iron overload.4,5 That, in combination with a malfunctioning spleen, may predispose patients to systemic infections by vascular tropic organisms like
Salmonella.
Several elements compounded the diagnostic difculty in
this patients case. He did not recall any recent symptoms of

The patient was given ceftriaxone after the Salmonella culture isolate proved susceptible to the antibiotic. Splenectomy
and subsequent initiation of specic drug therapy led to rapid
defervescence. A CT aortogram showed no mycotic aneurysms along the course of the thoracoabdominal aorta or its
immediate branches. Testing for human immunodeciency
virus antibody yielded negative results. He continued ceftriaxone treatment for 2 weeks from the date of splenectomy.
An uneventful recovery ensued, and he remained well 3
months after discharge.
This case illustrates that patients with Salmonella splenic
infection require a combination of surgical intervention and
antibiotic therapy for a complete cure.7 While total splenectomy remains the standard procedure, partial splenectomy has
also been successfully employed in pediatric patients with a
splenic abscess.8 The approach carries the advantage of
maintaining splenic function and reducing the risk of postsplenectomy infection from encapsulated bacteria, like
Streptococcus pneumoniae. Total splenic preservation with
percutaneous drainage also appears to be a promising treatment modality that deserves further evaluation.9

References
1. Tso AC, Roper DR, Wong CL, Bain B, Layton DM. Splenic infarction
in a patient with sickle cell trait and hereditary spherocytosis. Am J
Hematol. 2011;86:695-696.
2. Koduri PR, Vanajakshi S. Splenic infarction in thalassemia major. Ann
Hematol. 2013;92:573-574.
3. Jama AH, Salem AH, Dabbous IA. Massive splenic infarction in Saudi
patients with sickle cell anemia: a unique manifestation. Am J Hematol.
2002;69:205-209.
4. Cantinieaux B, Hariga C, Ferster A, De Maertelaere E, Toppet M,
Fondu P. Neutrophil dysfunctions in thalassaemia major: the role of cell
iron overload. Eur J Haematol. 1987;39:28-34.
5. Humbert JR, Winsor EL, Githens JM, Schmitz JB. Neutrophil dysfunctions in sickle cell disease. Biomed Pharmacother. 1990;44:153-158.
6. Warren MS, Gibbons RB. Left-sided pleural effusion secondary to
splenic vein thrombosis. A previously unrecognized relationship. Chest.
1991;100:574-575.
7. Ooi LL, Leong SS. Splenic abscesses from 1987 to 1995. Am J Surg.
1997;174:87-93.
8. De Greef E, Hoffman I, Topal B, Broers C, Miserez M. Partial laparoscopic splenectomy for splenic abscess because of Salmonella infection: a case report. J Pediatr Surg. 2008;43:E35-E38.
9. Faruque AV, Qazi SH, Arshad M, Anwar N. Isolated splenic abscess in
children, role of splenic preservation. Pediatr Surg Int. 2013;29:
787-790.