Long-term follow-up of subtotal splenectomy for hereditary spherocytosis: a single-center study

Pincez, Thomas; Guitton, Corinne; Gauthier, F.; Lambert, Guénolée de; Picard, Véronique; Fénéant-Thibault, Madeleine; Turhan, Ali G.; Mohandas, Narla; Tchernia, Gil; Garçon, Loïc

doi:10.1182/blood-2015-11-679357

Cited by 31 publications

(33 citation statements)

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“…HowellJolly bodies can also be used, but the method correlates poorly with other measurements. (34) .Pincez et al (34) showed a functional remnant spleen after subtotal splenectomy, in 96 % of their patients. Those patients underwent splenic scintigraphy at one year after surgery, and then every 5 years.…”

Section: Therapy Outcomesmentioning

confidence: 99%

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

Manciu¹,

Matei²,

Trandafir³

2017

chr

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RezumatSferocitoza ereditarã este o maladie congenitalã, caracterizatã prin anemie hemoliticã, ce afecteazã membranele eritrocitelor. Manifestãrile 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: sferocite, hemoliză, citometrie în flux, splenectomie, rezultate pe termen lung AbstractHereditary spherocytosis (HS) is a disease affecting the red blood cells membrane and belongs to the congenital hemolytic anemias. The clinical spectrum ranges from asymptomatic patients 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.

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Section: Therapy Outcomesmentioning

confidence: 99%

“…Pincez et al (34) showed that 82 % of their patients remained free of any transfusion after splenectomy. However, the hemolytic rate is not abrogated after splenectomy.…”

Section: Therapy Outcomesmentioning

confidence: 99%

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

Manciu¹,

Matei²,

Trandafir³

2017

chr

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“…Recently Pincez and colleagues have proposed subtotal splenectomy (residual splenic mass 15-25%) for severe and moderate HS. 68 They propose this approach in order to reduce long-term complications: after a long follow-up, subtotal splenectomy led to a good hematologic response in many patients, but 5 out of 79 patients remained transfusion dependent, 3 were considered as asplenic and 1 showed no significant increase of Hb. In our opinion, in severe HS cases total splenectomy would be preferred, while the role of subtotal splenectomy in symptomatic non-transfusion dependent patients needs to be evaluated case by case.…”

Section: Therapy and Managementmentioning

confidence: 99%

New insights on hereditary erythrocyte membrane defects

et al. 2016

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© Ferrata Storti Foundationmary role in the removal of aged RBCs. In order to perform these journeys RBCs must possess and maintain a significant deformability. The main author of this property is certainly the membrane, that ensures both mechanical stability and deformability. After the first proposed model of the RBC membrane skeleton 36 years ago, 1 containing the core elements of the modern model, many additional proteins have been discovered during the intervening decades, and their structures and interactions have been defined. RBC membrane structure has been extensively covered by excellent reviews.2,3 Herein we summarize the main concepts. RBC membrane is composed by a fluid double layer of lipids in which approximately 20 major proteins and at least 850 minor ones are embedded. 4 The membrane is attached to an intracellular cytoskeleton by protein-protein and lipid-protein interactions that confer the erythrocyte shape, stability and deformability. The transmembrane proteins have mainly a transporter function. However, several of these also have a structural function, usually performed by an intracytoplasmic domain interacting with cytoskeletal proteins.The lipid bilayer acts as a barrier for the retention of cations and anions within the red cells, while it allows water molecules to pass through freely. Human erythrocytes have high intracellular K + and low intracellular Na + contents when compared with the corresponding ion concentrations in the plasma. The maintenance of this cation gradient between the cell and its environment involves a passive outward movement of K + , which is pumped back by the action of an ATP-dependent Na + /K + pump in exchange for Na+ ions. This protein belongs to a class of transmembrane proteins with a transport function (Figure 1). The third and more important component of the RBC membrane is the cytoskeleton, a protein network that laminates the inner surface of the membrane. Spectrin aand β-chains, proteins 4.1, or 4.1R, and actin are the main components of this skeleton, maintaining the biconcave shape of the RBC. These components are connected to each other in two protein complexes; ankyrin and protein 4.1 complex. The former is composed by band 3 tetramers, Rh, RhAG, CD47, glycophorin A and protein 4.2. Whereas the protein 4.1 complex is composed by band 3 dimers binding adducins a-and β-, glycophorin C, GLUT1 and stomatin (Figure 1). The ends of spectrin tetramers converge toward a protein 4.1 complex (junctional complex). Electron microscopy (EM) shows that this latter links the tail of six spectrin tetramers, forming a pseudo-hexagonal arrangement. 5Spectrin tetramers include anion transporters (band 3 or chloride/bicarbonate exchange). The capability of these transporters to form aggregates could define the half-life of RBCs, causing antibody binding and removal by the spleen. Defects that interrupt this vertical structure (spectrin-actin interaction) underlie the biochemical and molecular basis of hereditary spherocytosis (HS), whereas defects in horizo...

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“…As the data in this area are mostly of an observational nature and limited to small case series, this is an entirely appropriate tool to summarize the existing literature in this area and communicate it in a manner that is understandable for readers . Most of the research in this area remains limited to several groups, including the leading work by Tchernia in France, Stoehr in Germany, and our own Splenectomy in Children With Congenital Hemolytic Anemia (SICHA) consortium in the United States …”

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confidence: 99%

“…2 Most of the research in this area remains limited to several groups, including the leading work by Tchernia in France, Stoehr in Germany, and our own Splenectomy in Children With Congenital Hemolytic Anemia (SICHA) consortium in the United States. [3][4][5][6][7][8][9] As with the design and interpretation of any systematic review, the first step is to outline important clinical questions. For spleen surgery, some of these questions are relatively straightforward, such as what are the expected hemoglobin and other hemolysis measures after surgery?…”

mentioning

confidence: 99%

Understanding splenectomy for children with hereditary spherocytosis

Rice

2016

Pediatric Blood & Cancer

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Long-term follow-up of subtotal splenectomy for hereditary spherocytosis: a single-center study

Cited by 31 publications

References 22 publications

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

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

New insights on hereditary erythrocyte membrane defects

Understanding splenectomy for children with hereditary spherocytosis

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