Thalassemia major is associated with which of the following features?

Thalassemia major is characterized by a significant imbalance in globin chain production due to mutations in the genes encoding for hemoglobin. In this condition, there is typically an inadequate production of one of the beta chains of hemoglobin, leading to ineffective erythropoiesis and a subsequent increase in red blood cell production as the body attempts to compensate for the severe anemia.

The red blood cell overproduction results from the bone marrow's response to chronic anemia, marked by high levels of erythropoietin. However, despite this overproduction, the red blood cells are often abnormal and do not function properly, contributing to chronic hypoxia. This combination leads to the clinical manifestations associated with the disease, including severe anemia, splenomegaly, and various complications due to iron overload from repeated blood transfusions required to manage the condition.

The other features mentioned are not characteristic of thalassemia major. For instance, patients typically do not have an increased platelet count as a direct feature of thalassemia; in fact, chronic disease often leads to thrombocytopenia due to hypersplenism. Normal levels of hemoglobin would not be expected in thalassemia major, as the condition is defined by significantly reduced hemoglobin levels