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Splenectomy in Homozygous Beta Thalassaemia: A Retrospective Study of 30 Patients
Author(s) -
Engelhard Dan,
Cividalli Gabriel,
Rachmilewitz Eliezer A.
Publication year - 1975
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1975.tb00870.x
Subject(s) - splenectomy , medicine , hemoglobinopathy , thalassemia , blood transfusion , beta thalassemia , surgery , retrospective cohort study , beta thalassaemia , pediatrics , hemolytic anemia , spleen
S ummary . In order to clarify the indications for splenectomy in patients with homozygous β thalassaemia we studied, retrospectively, the basal pre‐transfusion haemoglobin levels and blood transfusion requirements before and after splenectomy, in a series of patients with this disorder. Thirty‐six patients, of whom 20 underwent splenectomy, were included in this study. Three groups of patients with homozygous β thalassaemia were identified on the basis of clinical and laboratory findings. Two of the three groups consisted of 24 patients with β thalassaemia major, subdivided retrospectively according to their response to splenectomy. The third group consisted of six patients with β thalassaemia intermedia who had a comparably mild clinical course. In the first group of 16 patients with β thalassaemia major, monthly transfusions were required from the age of 6 months to 24 months and splenectomy resulted in temporary improvement for a period of 1–2 years. Thereafter, the transfusion requirements returned to pre‐operative levels with no sustained improvement in base‐line pretransfusion haemoglobin levels. Despite the fact that haematological improvement was temporary, it is felt that splenectomy is indicated in this group since the development and the general well‐being of the children was significantly improved. In the second group of eight patients with β thalassaemia major, transfusion requirements were relatively infrequent before 1–2 years, but after several years the transfusion requirement increased simultaneoulsy with the development of‘hypersplenism’. In these cases splenectomy was found to be beneficial for longer periods of time as manifested by a decrease in the transfusion requirement and an increase in the pretransfusion haemoglobin levels. Patients who developed hypersplenism relatively late in the course of their disease were usually the ones who derived long‐term benefit from splenectomy. In the third group of six patients with β thalassaemia intermedia and a benign clinical course, with higher basal haemoglobin levels and lower blood transfusion requirements, most of the splenectomies were performed because of mechanical pressure due to splenomegaly. After splenectomy, these patients showed continued improvement in their haemoglobin levels and decreased transfusion requirements.