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

Abstract

Hemoglobin Eb-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 Eb-thalassemia patients after splenectomy in terms of transfusion requirement, infections and other complications. Retrospective study conducted over a period of 3 years included 72 cases of splenectomized Eb-thalassaemia patients, considering decrease in transfusion requirements, new complications, antibiotic, anti-malarial prophylaxis and iron chelation therapy. Out of 1380 registered Eb-thalassemia patients, 618 (44.78 %) were regularly transfused and 72(5.22 %) underwent splenectomy. Mean age of diagnosis was 10.3 years. Nineteen patients (26.4 %) underwent splenectomy between 5 and 10 years, 38 cases (52.7 %) between 10 and 20 years. The leading cause (51.39 %) for splenectomy was mechanical discomfort. Mean steady state hemoglobin raised from pre-splenectomy level of 5.43-6.8 gm/dl after splenectomy. Mean transfusion requirement reduced from 18.1 to 7.8 units/year. Mean serum ferritin level increased from 907.58 to 1,091.6 ng/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.

Survival and complications in patients with haemoglobin E thalassaemia in Sri Lanka: a prospective, longitudinal cohort study

“…23 The threshold of liver iron concentration associated with poorer survival (5 mg/g) was lower than that in thalassaemia major (15 mg/g) 14 and similar to that in thalassaemia intermedia not involving haemoglobin E. 24 Two-thirds of patients underwent splenectomy. By contrast with previous findings, 25 in over half of these patients, splenectomy did not permanently mitigate the requirement for prescheduled transfusions. Although it might seem prudent to recommend avoidance of splenectomy in patients with haemoglobin E thalassaemia, shortages of blood throughout Asia complicate disease management, 2 such that steady-state haemoglobin concentrations sufficient to suppress splenic expansion are often not achieved.…”

Quality of life outcomes in thalassaemia patients in Saudi Arabia: a cross-sectional study

“…Although splenectomy is an effective measure to reduce transfusion requirements in thalassaemia, it does not prevent disfiguring skeletal changes, delayed growth and puberty, or chronic pain (28,29). Moreover, splenectomy increases the risk of infection and venous thromboembolism (28,29).…”

Modeling hemoglobin levels of patients chronically transfused with red blood cells

“…Hb endo is a calculated baseline concentration of native Hb reasonably expected to be bounded between a typical threshold trigger ($7 g/dL) and lower measured patient [Hb] ($4.5 g/dL). 11,12 This value is expected to vary for individual patients without transfusions and is a theoretical value while on transfusion therapy. If the patient stopped receiving transfusions and an equilibrium Hb level could be achieved safely, it is unlikely to equal to Hb endo due to the active biological processes that regulate iron and Hb levels.…”