In beta-thalassemia major, the primary mechanism of anemia is:

• A Excess alpha-chains precipitate within RBC precursors causing ineffective erythropoiesis and hemolysis ✓
• B Autoimmune destruction of RBCs by anti-Rh antibodies
• C Reduced hepcidin leads to iron overload without affecting hemoglobin synthesis
• D Deletion of alpha-globin genes reduces total hemoglobin content

Explanation

In beta-thalassemia, absent or reduced beta-chain synthesis leads to unpaired alpha-chains that are unstable and precipitate within erythroid precursors in the bone marrow, causing massive ineffective erythropoiesis (intramedullary destruction). Surviving RBCs also have Heinz body–like inclusions causing hemolysis in the spleen. Alpha-globin gene deletions characterize alpha-thalassemia.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.