[Current management of thalassemia intermedia.]

[alpha-thalassemia]

Thalassemia intermedia is a clinical entity where anemia is mild or moderate , requiring no or occasional transfusion . Non-transfusion-dependent thalassemia encompasses 3 main clinical forms : beta-thalassemia intermedia , hemoglobin E /beta-thalassemia and alpha-thalassemia intermedia (HbH disease ). Clinical severity of thalassemia intermedia increases with age , with more severe anemia and more frequent complications such as extramedullary hematopoiesis and iron overload mainly related to increased intestinal absorption . Numerous adverse events including pulmonary hypertension and hypercoagulability have been associated with splenectomy , often performed in thalassemia intermedia patients . The potential preventive benefit of transfusion and chelation therapies on the occurrence of numerous complications supports the strategy of an earlier therapeutic intervention . Increasing knowledge about pathophysiological mechanisms involved in thalassemia erythropoiesis and related iron overload is currently translating in novel therapeutic approaches .