Which among the following is not a feature of Mediterranean anemia?

 # Which among the following is not a feature of Mediterranean anemia?

A. Rodent like facies

B. Fessa bodies

C. Rib within rib appearance

D. Platybasia

The correct answer is D. Platybasia.

Mediterranean anemia, also known as thalassemia, is a group of inherited blood disorders characterized by reduced or absent production of normal hemoglobin. This leads to various clinical manifestations, including skeletal changes due to bone marrow expansion. However, platybasia (flattening of the skull base) is not typically associated with thalassemia.

Rodent-like facies: This is a characteristic of sickle cell anemia, not thalassemia. It refers to the overgrowth of the maxillary bones and protrusion of the upper incisors.

Fessas bodies: In heterozygotes, the disease is mild and is called thalassemia minor or thalassemia trait. It represents both α-and β-thalassemia. Homozygotes may exhibit a severe form of the disease that is called thalassemia major or homozygous -thalassemia, in which the production of β-chains is markedly decreased or absent, and a consequent decrease in synthesis of total hemoglobin occurs. This results

in severe hypochromic anemia. Furthermore, excess α-chains, which synthesize at the normal rate, precipitate as insoluble inclusion bodies within the erythrocytes and their precursors.

The presence of such intracellular inclusion bodies (Fessas bodies) leads to increased erythrocyte hemolysis and severe ineffective hematopoiesis. Approximately 70–85% of marrow normoblasts are destroyed in severely affected patients. These processes result in profound anemia and an associated increase in marrow activity, which is estimated to increase 5- to 30-fold.

Rib within rib appearance: This is also known as "rib notching" and is caused by bone marrow expansion eroding the undersurface of the ribs.

Reference:

Radiopaedia: https://radiopaedia.org/articles/thalassaemia