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Selective inhibition of JAK2-driven erythroid differentiation of polycythemia vera progenitors.

Patients with beta-thalassemia intermedia should receive as few transfusions as possible to avoid iron overload. However, suppression of abnormal hematopoiesis by periodic red blood cell transfusion may be valuable in severely affected patients. In beta thalassemia major, give transfusions as needed to maintain the hemoglobin level around 9 to 10 g/dL (90 to 100 g/L) and avoid severe clinical manifestations.

To prevent or delay complications due to iron overload, excess (transfusional) iron must be removed (eg, via chronic iron chelation therapy). Chelation therapy is generally initiated when serum ferritin levels are > 1000 ng/mL (1000 mcg/L) or after about 1 to 2 years of scheduled transfusions. Splenectomy may help decrease transfusion requirements for patients with significant splenomegaly.

Allogeneic stem cell transplantation is the only curative option and should be considered in all patients.
Thalassemias result from decreased production of at least one globin polypeptide chain (beta, alpha, gamma, delta); the resultant abnormal red blood cells are microcytic, often abnormally shaped, and prone to hemolysis (causing anemia).
Splenomegaly, often massive, is common and can result in splenic sequestration that accelerates destruction of red blood cells (including transfused ones).
Iron overload is common because of increased absorption (due to defective erythropoiesis) and frequent transfusions.
Diagnose using hemoglobin electrophoresis.
Transfuse as needed, but monitor for iron overload and use chelation therapy.
Splenectomy may help decrease transfusion requirements for patients with splenomegaly.
Allogeneic stem cell transplantation is curative.