Thalassemia ______ individuals will develop hemolysis, splenomegaly, bone marrow hyperplasia and bone marrow deformities.

Thalassemia ______ individuals will develop hemolysis, splenomegaly, bone marrow hyperplasia and bone marrow deformities.

• A. Major
• B. Minor
• C. Intermedia
• D. Silent carrier

Answer: (A)

In thalassemia major there is a severe deficiency of beta-globin, causing marked chronic anemia and a strong erythropoietic drive. The body compensates by expanding the bone marrow (bone marrow hyperplasia), which leads to skeletal deformities from marrow crowding, especially in facial bones. Hemolysis and increased destruction of abnormal red cells stimulate the spleen, resulting in splenomegaly. This combination of ongoing hemolysis, splenic enlargement, and marrow expansion with deformities is characteristic of thalassemia major, whereas thalassemia minor or silent carriers have much milder disease or no symptoms at all, and intermedia lies between the two.

In thalassemia major there is a severe deficiency of beta-globin, causing marked chronic anemia and a strong erythropoietic drive. The body compensates by expanding the bone marrow (bone marrow hyperplasia), which leads to skeletal deformities from marrow crowding, especially in facial bones. Hemolysis and increased destruction of abnormal red cells stimulate the spleen, resulting in splenomegaly. This combination of ongoing hemolysis, splenic enlargement, and marrow expansion with deformities is characteristic of thalassemia major, whereas thalassemia minor or silent carriers have much milder disease or no symptoms at all, and intermedia lies between the two.