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Pathophysiology of beta thalassemia

Author
Stanley L Schrier, MD
Section Editors
William C Mentzer, MD
Donald H Mahoney, Jr, MD
Deputy Editor
Jennifer S Tirnauer, MD

INTRODUCTION

Beta thalassemia is an inherited hemoglobinopathy in which the production of one or both beta globin chains is impaired. The clinical manifestations include hemolytic anemia and impaired iron handling, the severity of which depends on the degree of impairment in beta globin production.

This topic reviews the pathophysiology of anemia in beta thalassemia, which includes ineffective erythropoiesis and hemolysis. Related issues are discussed in separate topic reviews:

Thalassemia genetics – (See "Molecular pathology of the thalassemic syndromes".)

Thalassemia laboratory diagnosis – (See "Methods for hemoglobin analysis and hemoglobinopathy testing".)

Beta thalassemia:

                   

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Literature review current through: Aug 2016. | This topic last updated: Aug 9, 2016.
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