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Host genetic variation

Prognosis

Life expectancy is normal for people with beta-thalassemia minor or alpha-thalassemia minor. The prognosis of Hb H disease and beta-thalassemia intermedia varies.

Life expectancy is decreased in people with beta-thalassemia major mostly due to complications from chronic transfusions.

Treatment

  • Often red blood cell transfusion, with or without iron chelation therapy
  • Splenectomy if splenomegaly is present
  • Allogeneic stem cell transplantation if possible

In patients with alpha-thalassemia trait or beta-thalassemia trait, no treatment is needed.

In Hb H disease, splenectomy may be helpful if anemia is severe or splenomegaly is present.

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