Beta-thalassemia major patients require regular transfusions starting in infancy because the predominant hemoglobin in adult life (HbA = α2β2) cannot be synthesized. Splenomegaly in these patients is primarily attributable to:

• A Splenic sequestration of sickled red cells
• B Extramedullary hematopoiesis in the spleen ✓
• C Iron deposition causing congestive splenomegaly
• D Lymphoid hyperplasia secondary to recurrent infections

Explanation

In beta-thalassemia major, the profound anemia drives expansion of erythropoiesis beyond the bone marrow capacity, resulting in extramedullary hematopoiesis in the spleen, liver, and other sites, causing massive splenomegaly ('work hypertrophy'). Splenic sequestration of sickle cells is the mechanism in sickle cell disease. Iron deposition (hemosiderosis) is a complication of repeated transfusions but is not the primary cause of splenomegaly.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.