THE ROLE OF SURGERY IN HOMOZYGOUS β-THALASSAEMIA
AbstractBackground: Various Strategies are adopted for the control and management of thalassaemia. Despite advances in themedical management of thalassaemia there remains a definite place for splenectomy in the management ofthalassaemia, particularly in a developing country like Pakistan. Methods: The Surgical aspects of the management ofpatients with homozygous (3-thalassaemia are reviewed, and operative findings in 15 patients with the disease whowere subjected to splenectomy are described. Results: Post-operatively frequency of blood transfusion was reducedfor maintaining the haemoglobin level, due to decreased blood consumption. The age range was from 12-13 years.Hepatomegaly was present in all the cases. In 73 percent cases hyperplastic lymph nodes containing erythropoieticelements were found. Cholelithiasis was seen in only one case. The recovery' was smooth in 10 (67%) cases. Woundinfection developed in 2 cases. Pneumonia occurred in only one case. Two patients developed fever and one itemswhich responded to conservative treatment.
Khattak FM, Saleem M. Prevalence of heterozygous Pthalassaemia trait in northern areas of Pakistan. J. Pak.
Med. Associ. 1992; 42: 32-34.
Olivieri NF. The p-thalassaemia: A review article. Med.
Progress 1999; 341(2): 99-109.
Eraklis AJ, Kevy SV, Diamond LK. Hazard of
overwhelming infection alter splenectomy in
childhood. New. Eng. J. Med. 1967; 276:1225-29.
Craig RP, Bate CM and Humphries G. Some surgical
aspects of homozygous p-thalassaemia. Bull. Cyprus
Surg. Soc. 1975; 277-80.
Marangos GN. Splenectomy in patients with
thalassaemias. Bull Cyprus Surg. Soc (English Ed)
Dewey KW, Grossman H and Canale VC.
cholelithiasis in thalassaemia major. Radiology 1970;
Ficara BJ. Surgical problems in splenectomy for
Cooley’s anaemia. Ann. NY. Acad. Sci, 1964: 119: