Does Profile of Hemoglobin Eβ-thalassemia Patients Change After Splenectomy? Experience of a Tertiary Thalassemia Care Centre in Eastern India.

Abstract

Hemoglobin Eβ-thalassemia is by far the commonest form of thalassemia intermedia. Its phenotype ranges from mild anemia to severe transfusion-dependency necessitating splenectomy in many patients. The present study aimed to systematically analyze both clinical as well as laboratory parameters in profile of Eβ-thalassemia patients after splenectomy in terms of transfusion requirement, infections and other complications. Retrospective study conducted over a period of 3years included 72 cases of splenectomized Eβ-thalassaemia patients, considering decrease in transfusion requirements, new complications, antibiotic, anti-malarial prophylaxis and iron chelation therapy. Out of 1380 registered Eβ-thalassemia patients, 618 (44.78%) were regularly transfused and 72(5.22%) underwent splenectomy. Mean age of diagnosis was 10.3years. Nineteen patients (26.4%) underwent splenectomy between 5 and 10years, 38 cases (52.7%) between 10 and 20years. The leading cause (51.39%) for splenectomy was mechanical discomfort. Mean steady state hemoglobin raised from pre-splenectomy level of 5.43-6.8gm/dl after splenectomy. Mean transfusion requirement reduced from 18.1 to 7.8units/year. Mean serum ferritin level increased from 907.58 to 1,091.6ng/ml. Post-splenectomy; 21 (29.17%) patients developed facial deformities, 17 (23.6%) delayed pubertal growth, 11 (15.28%) venous thromboembolism, five (6.94%) pulmonary hypertension and four (5.5%) had extramedullary hematopoiesis. Five (6.96%) patients had documented bacterial infections and two (2.78%) suffered from malaria. Forty eight patients (66.67%) started with iron chelation therapy; but majority (52.7%) stopped. Major advantage of splenectomy is reduced transfusion requirement, though it cannot prevent skeletal abnormalities and delayed pubertal growth. In resource constraint countries like India, routine anti-malarial and antibacterial prophylaxis is not desirable; iron chelation therapy should be encouraged and ensured.