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Previous Volume 353:1135-1146 September 15, 2005 Number 11 Next

-Thalassemia

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Thalassemia is a hereditary anemia resulting from defects in hemoglobin production.¹ -Thalassemia, which is caused by a decrease in the production of -globin chains (Figure 1), affects multiple organs and is associated with considerable morbidity and mortality.² Accordingly, lifelong care is required,³ and financial expenditures for proper treatment are substantial.⁴

View larger version (52K): [in this window] [in a new window]   Figure 1. Management of Thalassemia and Treatment-Related Complications. The anemia that is associated with thalassemia may be severe and is accompanied by ineffective erythropoiesis, with bone expansion and extramedullary hematopoiesis in the liver, spleen, and other sites, such as paravertebral masses. Transfusion therapy, which is the mainstay of treatment, allows . . . [Full Text of this Article]

 
Molecular and Cellular Pathology

Clinical Manifestations and Supportive Therapy

Anemia and Transfusion Therapy

Endocrinopathies and Bone Disease

Iron Overload — Pathogenesis, Measurement, and Therapy

Hypercoagulability

Hematopoietic Stem-Cell Transplantation

Experimental Therapies

Cellular and Molecular Modifiers

Potential Role of Antioxidants

Experimental Molecular Therapies

Progress in Prenatal Diagnosis

Treatment in the Developed versus the Underdeveloped World

Source Information

From the Hematology Department, Hebrew University–Hadassah Medical Center, Ein Kerem, Jerusalem (D.R.); and the Hematology Department, E. Wolfson Medical Center, Holon (E.R.) — both in Israel.

Address reprint requests to Dr. Rund at the Hematology Department, Hadassah University Hospital, Ein Kerem, Jerusalem IL91120, Israel, or at rund@cc.huji.ac.il.

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