Partial splenectomy in homozygous beta thalassaemia.

Abstract

Hypersplenism is a very common complication in homozygous ,B thalassaemia. In regularly transfused patients it is suspected before the increased blood consumption and is sometimes associated with thrombocytopenia or leucopenia, or both. A high blood transfusion regimen usually delays this complication,' though it doesn't always suppress it. Splenectomy allows for the reduction of transfusional blood requirements thus reducing iron overload.2 However, this operation exposes patients, who are mainly under 5 years of age (but also older children and even adults), to the risk of overwhelming postsplenectomy infections.3 ' The severity of these infections has led to the search for alternative surgical techniques to replace total splenectomy so that blood requirements are reduced but the splenic contribution to the host defence against infections is preserved.We report our experience with 30 thalassaemic patients who underwent one of these alternative techniques, partial splenectomy.Patients and methods Thirty patients affected with homozygous ,B thalassaemia were included in the study; 24 had thalassaemia major and six thalassaemia intermedia. The diagnosis of thalassaemia major and intermedia was based on transfusion requirements.5 The patients' mean (SD) age was 7-9 (3 7) years (range 3-19 years) at the time of the partial splenectomy. Twenty five patients received a low transfusion regimen and five received a high transfusion regimen (table 1).In all patients about nine tenths of the total hypertrophied splenic mass was ablated at operation. The

Outcome of Partial Splenectomy for Hypersplenism in Children with Thalassemia

“…Intravenous fluid, injection penicillin, paracetamol suppository and other supportive therapy were administered to treat (OPSI). de Montalembert (1990) 12 found no infection after partial splenectony in six children which is consistent with our study. The less incidence of postsplenectomy infection in the partial group in our study indicates that the immune and the phagocytic function of the spleen were preserved.…”

“…13 In order to obviate this problem it has been thought that partial splenectomy may offer a better balance between palliation and retention of some host defense against the aforesaid infection. 12 It is considered that partial splenectomy is a safe and better alternative to total splenectomy for palliation of thalassemia patients when splenectomy is indicated. Therefore this study has been designed to compare the short term outcomes of partial and total splenectomy in thalassemia patients to testify the above proposition.…”

“…So, autotransplantation or the preservation of splenenculi after total splenectomy has been practiced to prevent post splenectomy sepsis. [11][12] Total splenectomy has been practiced for a long time for palliation for thalassemic children. Conceivably it provides the best opportunity for palliation in thalassemic children by removing a substantial portion of reticuloendothelial system.…”

Effect of subtotal splenectomy for management of hereditary pyropoikilocytosis

“…Hereditary elliptocytosis is a group of disorders that is heterogeneous in the severity of haemolysis, ranging from an asymptomatic carrier state to a severe haemolytic anaemia, especially in hereditary pyropoikilocytosis (HPP) (Gallagher, 2004). Subtotal splenectomy has been proposed in the management of some inherited red blood cell (RBC) disorders in order to decrease the haemolytic rate while preserving the phagocytic function of the splenic remnant (de Montalembert et al , 1990; Sandoval et al , 1997; Idowu & Hayes‐Jordan, 1998 ; Bader‐Meunier et al , 2001). We have already reported the beneficial effects of subtotal splenectomy for management of hereditary spherocytosis (HS) (Bader‐Meunier et al , 2001).…”

Partial splenectomy followed by total splenectomy in thalassemia

“…It has been practiced for the same indication in some centers of the world. 1 During the six months follow-up period in a study in children with thalassemia showed favorable initial results. 2 After six years of partial splenectomy, one boy of this study group presented to us for total splenectomy, at BSMMU, again with increasing need of too much blood transfusions, secondary hypersplenism and mechanical discomfort with huge splenomegaly.…”