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Review Article

Chirurgia (2017) 112: 110-116


No. 2, March - April
Copyright© Celsius
http://dx.doi.org/10.21614/chirurgia.112.2.110

Hereditary Spherocytosis – Diagnosis, Surgical


Treatment and Outcomes. A Literature Review
Simona Manciu1,2, Emil Matei1,2, Bogdan Trandafir2
1
Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
2
Department of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania

Corresponding author: Rezumat


Bogdan Trandafir, MD
Department of General Surgery
and Liver Transplantation, Fundeni
Clinical Institute, Bucharest, Romania Sferocitoza ereditarã este o maladie congenitalã, caracterizatã prin
E-mail: bogdan21t@gmail.com anemie hemoliticã, ce afecteazã membranele eritrocitelor. Manifes-
tãrile clinice variazã de la forme aproape asimptomatice la forme
severe, ce necesitã transfuzii. Diagnosticul se bazeazã pe examenul
clinic, hemograma cu numãrul reticulocitelor, prezenţa unui istoric
familial şi teste de laborator specific, cum ar fi testul EMA (eosin-5-
maleimide binding test) sau testul AGLT (Acidified Glycerol Lysis
Time). Splenectomia este consideratã tratamentul standard în
formele moderate pânã la severe de boalã. Totuşi, este cunoscut
faptul cã splenectomia totalã expune pacientul la complicaţii
infecţioase, uneori letale, ceea ce a determinat indicarea acestei
metode cu precauţie. Astfel splenectomia subtotalã sau parţialã
a devenit o alternativã de tratament fezabilã. Aceasta reduce
distrugerea eritrocitelor, în timp ce conservã funcţia imunã a splinei.
Majoritatea studiilor au arãtat beneficii certe, pe termen scurt, dupã
splenectomie, în cazul acestor pacienţi. Totuşi, pânã în prezent,
rezultate pe termen lung certe, adicã pe o perioadã de peste 5 ani de
urmãrire postoperatorie, încã lipsesc.

Cuvinte cheie:

Abstract
Hereditary spherocytosis (HS) is a disease affecting the red blood
cells membrane and belongs to the congenital hemolytic ane-
Received: 27.02.2017
Accepted: 21.03.2017 mias. The clinical spectrum ranges from asymptomatic patients

110 Chirurgia, 112 (2), 2017


Hereditary Spherocytosis – Diagnosis, Surgical Treatment and Outcomes. A Literature Review

to severe forms requiring transfusions in early childhood. The diagnosis can be based on the
physical examination, complete red blood cell count, reticulocytes count, medical history and
specific tests, preferentially the EMA test (eosin-5-maleimide binding) test and AGLT (Acidified
Glycerol Lysis Time). Splenectomy is considered the standard surgical treatment in moderate
and severe forms of hereditary spherocytosis. Total splenectomy exposes the patient to a life -
long risk of potentially lethal infections and thus, its usage was reconsidered. Because of this
reason, a feasible alternative is the partial splenectomy. The use of partial splenectomy aims to
retain splenic immunologic function, while at the same time to decrease the rate of hemolysis.
The long - term outcomes of patients with total or subtotal splenectomy for congenital hemolytic
anemia, still remain unclear, but the majority of the studies showed a qualitative resolution of anemia
and reduction of transfusion rate. Despite the well known advantages of conservative surgery, the
optimal choice of treatment and outcomes should be confirmed with the patient.

Key words: spherocytes, hemolysis, flow cytometry, splenectomy, long-term outcomes

Introduction of all people affected by hereditary spherocyto-


sis suffer from an autosomal-dominant trait,
Hereditary spherocytosis (known as Minkowski- while only 15 percent have an autosomal-reces-
Chauffard disease) is a heterogeneous group of sive inheritance. Some patients acquire the
diseases affecting the red blood cells (1). disease on account of new mutations.(2)
Clinically, the disease manifests itself depending
on the various mutations of genes that encode Clinical presentation
membrane proteins, their various functional
consequences, and the mode of inheritance. (2)
Hereditary spherocytosis belongs to the
congenital hemolytic anemias, named after The clinical spectrum of hereditary spherocy-
the microscopic aspect of erythrocytes (the cell tosis ranges from asymptomatic patients, with
does not have the usual shape, i.e. spherocyto- incidental diagnosis by laboratory analysis or
sis means more sphere-shaped), and this other indication, to severe forms requiring
change also makes it more fragile than usual, transfusions in early childhood. (2)
causing its rupture. (3) The characteristic features in hereditary sphe-
In 1900, Oskar Minkowski was the first to rocytosis are anemia, jaundice, splenomegaly and
describe this disease and published his obser- family history. The typical complications of the
vations on familial clusters. (4) disease are those related to cholelithiasis, a
consequence of chronic hemolysis and aplastic
crisis more frequent after initial infection with
Parvovirus B19). (1) The aplastic crisis might
Commonly, the cause of hereditary spherocyto- lead to dramatic decrease of the hemoglobin
sis is represented by membrane defects. These concentration and, in this case, a blood transfu-
defects decrease the deformability of the sion is mandatory. (7)
erythrocytes and accelerate their degradation Another complication that might appear in
in the spleen (5). The most frequently affected the mild form of the disease (in patients without
genes are those encoding the membrane pro- splenectomy) is the extramedullary hemato-
teins ankyrin, band 3, and spectrin. (6) poiesis, with the clinical picture of intrathoracic,
Modifications of the genes encoding protein 4.2, paravertebral tumors. Elderly patients may also
the RH complex and cases with so far unde- exhibits varicose ulcers. (8)
fined defects are less frequent. Seventy percent The association between hereditary sphero-

Chirurgia, 112 (2), 2017 111


S. Manciu et al

cytosis and spinocerebellar ataxia was reported possible). The test is reserved to exceptional
in few patients, but it is not known yet whether cases (the diagnosis cannot be obtained other-
this association is based on the same genetic wise), as it can be performed only with fresh
defect. (9) blood taken at the site of analysis.

Diagnosis

A rational diagnostic can be based on physical This analysis can be used both as a quantitative
examination, complete blood cell count, reticulo- method (by means of gel electrophoresis in order
cyte count etc. No single test identifies all forms to prove a decrease in the number of membrane
of hereditary spherocytosis, and that is why it is proteins) and as qualitative method (in order to
recommended to combine two test procedures identify the affected proteins). However, these
(preferentially the EMA test and AGLT). The methods do not contribute too frequently to
typical procedures are found below. (10) diagnosis.

The acidified glycerol lysis test (AGLT) is a This analysis identifies the genetic defect
highly specific method for measuring hemo- specific to the patient and/ or the family, but it
lysis. The sensitivity of the test is 80 - 95%. The remains reserved to special cases because of the
test must be performed within 24 hours after numerous target genes showing heterogeneity
blood sampling or by using samples dispatched of possible mutations and the considerable
by courier (depending on the season, samples related costs.
must be chilled). (11)
The role of splenectomy in hereditary spherocytosis

Splenectomy is considered the standard surgical


This method or the EMA test was introduced treatment in moderate and severe forms of
quite recently (2000).(11) It is based on the bind- hereditary spherocytosis. It is indicated for
ing of the fluorescent dye eosin-5-maleimide to patients (particularly children), with recurrent
erythrocytes. The binding is decreased in hemolytic crisis, significant splenomegaly,
patients with hereditary spherocytosis, as com- severe aplastic crisis, cholelithiasis and develop-
pared with healthy people. (12) The sensitivity mental delay. These symptoms and signs are
of the tests is 90 - 95%, while its specificity is recognized to be caused by hemolysis of imma-
95 - 99%. The result will be valid only if the ture reticulocytes undergoing conditioning in
measurement proceeds within a maximum the spleen.
dwell time of 48 hours between blood sampling The degree of splenomegaly is the indicator
and test performance. The binding is increased of the severity of anemia and hemolysis. In
in cases of stomatocytosis, a hereditary defor- this case, the surgical approach remains the
mation of the erythrocytes which are swollen most attractive option.
and cup-shaped, causing congenital hemolytic Except for the unusual autosomal - recessive
anemia. (13) variant of hereditary spherocytosis, splenectomy
usually eliminates hemolysis and the associated
signs and symptoms (15). However, splenectomy
does not correct the cytoskeletal membrane
Currently, ektacytometry is a method available defects of hereditary spherocytosis. Thus the
only at quite a few sites. The osmotic-gradient erythrocytes are still spheroidal - shaped, with a
ektacytometry determines exactly the osmotic disorganized membrane ultrastructure, but they
fragility; (therefore, the differentiation between are larger (16).
spherocytosis and macrocytic stomatocytosis is The surgical management of hereditary

112 Chirurgia, 112 (2), 2017


Hereditary Spherocytosis – Diagnosis, Surgical Treatment and Outcomes. A Literature Review

Table 1. Classification of spherocytosis and indications for splenectomy (modified from Bolton-Maggs et al 2011(1);
Eber et al, 1990 (14) (copyright 1990 Elsevier)

Trait Mild Moderate Severe


Haemoglobin (g/l) normal 110-150 80-120 60-80
Reticulocyte count, % normal (< 3%) 3-6 >6 > 10
Bilirubin (lmol/l) < 17 17-34 > 34 > 51
Spectrin molecules per erythrocyte
(% of normal)* 100 80-100 50-80 40-60
Splenectomy No required Usually not necessary Necessary during Necessary
during childhood school age before (delay until 6
puberty years if possible)

spherocytosis presumed, historically, total Several studies have set the minimal
splenectomy. However, total splenectomy amount of the residual parenchyma necessary
exposes the patient to a life - long risk of to maintain adequate phagocytic function, to
potentially lethal infections (17) and thus, its approximately 15% of the enlarged spleen.
usage was reconsidered. In children less than (25-27)
5 years old, the risk of overwhelming post- Technically, a splenectomy can be per-
splenectomy sepsis may be increased 60- to formed either in the traditional open manner
100 fold, compared with that of children with- or with a minimally invasive approach.
out a splenectomy (18) In an open surgery, a partial splenectomy can
There are also concerns that despite the be safely performed preserving either the upper
immunizations against encapsulated bacteria (28) or the lower pole of the spleen. (26, 29)
and the postoperative antibiotic prophylaxis, Vasilescu et al (24,30) have shown that
the risk after total splenectomy is never preservation of the lower pole of the spleen
eliminated. (17,19,20) Because of this reason, allows a better mobilization of the splenic
a number of alternatives to total splenectomy remnant, and a more accurate assessment of
have been considered, including auto-trans- the volume. The length of the splenic remnant
plantation of the removed spleen and partial ranged between 3.5 and 6 cm, depending on the
splenectomy. (21) initial size of the spleen. In the same study,
The use of partial splenectomy, particularly it was shown that the 15% target was more
in pediatric patients, aims to retain splenic difficult to be achieved using the laparoscopic
immunologic function, while at the same time to approach, compared with the open surgery, and
decrease the rate of hemolysis. Partial splenec- the evaluation of the remnant spleen was more
tomy is associated with higher antibody titers challenging when the upper pole was preserved.
after immunization and better pneumococcal Based on the Vasilescu and co-workers
splenic uptake, in comparison to splenic auto- experience in laparoscopy (over 530 minimally
transplantation. (22-24) invasive splenectomies), over 42 minimally

Table 3. Splenectomies for hereditary spherocytosis *


(Fundeni Clinical Institute 2002 -2015)
Table 2. Splenectomies for hemolytic anemia Hereditary spherocytosis
*(Fundeni Clinical Institute experience 2002 -2015)
Children Adults
Hemolytic anemia Open approach 29 No patients 46 17
No patients -115 Laparoscopic approach 30 Subtotal splenectomies 31 11
Robotic approach 26 Total splenectomies 15 6

Chirurgia, 112 (2), 2017 113


S. Manciu et al

invasive subtotal splenectomies were per- regarded with caution because of its intrinsic
formed in patients with hereditary micro- limitations of a single center with a retrospec-
spherocytosis, preserving either the upper or tive design. (24)
the lower splenic pole. The majority of the studies from the litera-
However, nowadays, the minimally invasive ture showed a qualitative resolution of anemia
approach has become more attractive and popu- and decreased number of reticulocytes. This
lar, and in most of the experienced centers it is was a direct result of an increase in the red
considered the standard of care, because of the blood cell lifespan, with values far from
important advantages. Thus shorter hospital normal. In children younger than 6 years old
stay, less postoperative adverse events and bet- with severe disease, the procedure reduces the
ter cosmetic outcomes are the main advantages transfusion rate and increases the hemoglobin
of a minimally invasive approach. (31) to a level compatible with normal growth and
The choice of the blood vessels to be pre- activity. Those benefits varied according to the
served in order to supply the splenic remnant disease severity. Furthermore, the need for
represents a matter of debate. Thus there are blood transfusions is significantly reduced.
mainly two options : to preserve a short gastric This eliminates the associated risks of blood-
artery (32) or a branch of the left gastro - borne viral infections and iron overload.
epiploic artery (33). However, in some patients Pincez et al (34) showed that 82 % of their
the short gastric vessels are too small and too patients remained free of any transfusion
scant or their anatomy is abnormal. after splenectomy. However, the hemolytic
Thus if there is no possibility to use the short rate is not abrogated after splenectomy.
gastric vessels or the splenic vessels, the con- As previously reported, the bilirubin level
version to a total splenectomy is mandatory. may remain unchanged in some cases, when
Rogulski et al reported 3 patients (out of 15) patients did not undergo cholecystectomy at
with intraoperative splenectomy totalization the time of splenectomy. Residual hemolysis
due to the lack of blood supply and ischemia. may increase the risk of gallstone formation
Despite of its technical difficulties, laparos- and some patients may develop cholelithiasis
copic subtotal splenectomy is considered the during the follow-up.
standard surgical treatment in many centers. In the case of a partial splenectomy, an
The laparoscopic approach is safe and effective, important concern is the regrowth of the
while the resection only of a portion of the remnant, consistently noted in many previous
spleen, provide the limitation of the effects of reports (25,33,35). This disadvantage appears to
severe hemolysis combined with the integrity of be more frequently encountered in young
the innate immune system. children. Nevertheless, partial splenectomy
should be considered for those particular
Therapy outcomes patients, because of the immaturity of the
immune system. Thus, those patients are not
The long - term outcomes of patients with able to make anti - polysaccharide antibodies in
total or subtotal splenectomy for congenital response to encapsulated bacteria.
hemolytic anemia, still remain unclear. The After a total splenectomy, the risk of
reason is that long - term results are still septicemia associated with meningitis can be
lacking. Pincez et al reported in 2016 (34), a as high as 5%, with a mortality risk of 1 – 5 %.
series of 79 patients from 71 families who (7, 36). Therefore, the indications for a total
underwent subtotal splenectomy for hereditary splenectomy should be restraint to very few
spherocytosis. To date, this is the largest data- cases, while a conservative surgery should be
base of partially splenectomized patients for the first option for the largest part of the
hereditary spherocytosis, with the longest patients.
follow-up (mean follow-up time - 11 years). The phagocytic function of the remnant
However, the results of this study should be spleen can be assessed by scintigraphy, which

114 Chirurgia, 112 (2), 2017


Hereditary Spherocytosis – Diagnosis, Surgical Treatment and Outcomes. A Literature Review

Table 4. The most important studies about hereditary spherocytosis and surgical treatment [after Guizzetti (37)]

Study Study design Sample size Period Splenectomy and procedure


Rice et al.(38) Prospective cohort 16 1993-2000 PS, open
Rice et al. (39) Retrospective cohort 19 2005-2013 PS,mixed
Rescorla et al.(40) Prospective cohort 111 1995-2006 TS (mostly), laparoscopy
Wood et al.(41) Institutional case series 70 2000-2008 TS, laparoscopy (mostly)
Mariani et al.(42) Institutional case series 121 1980-2008 TS, open
Abdullah et al.(15) Retrospective cross-section 1657 1988-2004 TS, open

must show radionuclide uptake in the splenic sis. The Lancet 372: 1411–1426, 2008.
3 Inati A, Noun P, Kabbara N, Salloum C, Kmeid M, Sadek M, Abbas
area. However, pitted erythrocytes, measured HA and Kahale M: A multicenter study on the Lebanese experience
by phase microscopy, is considered the gold with hereditary spherocytosis. Pediatr Blood Cancer 61:
standard method and is widely available. The 1895–1896, 2014.
4 Kar R, Rao S, Srinivas UM, Mishra P and Pati HP: Clinico-hemato-
normal clinical range is 0 – 4 %, and in case of logical profile of hereditary spherocytosis: experience from a terti-
splenomegaly it may increase to 70 %. Howell- ary care center in North India. Hematology 14: 164–167, 2013.
5 Delaunay J: The molecular basis of hereditary red cell membrane
Jolly bodies can also be used, but the method disorders. Blood Rev 21: 1–20, 2007.
correlates poorly with other measurements.(34) 6 Da Costa L, Galimand J, Fenneteau O and Mohandas N: Hereditary
.Pincez et al (34) showed a functional remnant spherocytosis, elliptocytosis, and other red cell membrane disor-
ders. Blood Rev 27: 167–178, 2013.
spleen after subtotal splenectomy, in 96 % of 7 Vasilescu C, Stanciulea O and Tudor S: Laparoscopic versus robot-
their patients. Those patients underwent ic subtotal splenectomy in hereditary spherocytosis. Potential
splenic scintigraphy at one year after surgery, advantages and limits of an expensive approach. Surg Endosc 26:
2802–2809, 2012.
and then every 5 years. Vasilescu et al (7) 8 Buesing KL, Tracy ET, Kiernan C, Pastor AC, Cassidy LD, Scott JP,
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Journal of Pediatric Surgery 45: 1682–1686, 2010.
Conclusion 10 Eber SW, Pekrun A, Neufeldt A and Schröter W: Prevalence of
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