Thalassemia: the consequences of unbalanced hemoglobin synthesis

DG Nathan, RB Gunn - The American journal of medicine, 1966 - Elsevier
Thalassemia major or Cooley's anemia is a severe, usually fatal form of inherited anemia.
Beginning approximately two to four months after birth, the disease is associated with
profound anemia, jaundice, splenomegaly, expanded marrow space, siderosis and
cardiomegaly. The peripheral smear is characterized by extreme poikilocytosis, anisocytosis
and anisochromia. Target cells and nucleated erythroid precursors abound. Transfusion
therapy is mandatory in most cases. Splenectomy may be necessary if marked …