β-thalassemia intermedia: a clinical perspective.

Ali T Taher, Eliezer A Rachmilewitz, Khaled M Musallam

Journal: Cold Spring Harbor perspectives in medicine 2013;2(7):a013482

PMID: 22762026

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Abstract

Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with β-thalassemia intermedia has substantially increased over the past decade. Earlier studies observed that patients with β-thalassemia intermedia experience a clinical-complications profile that is different from that in patients with β-thalassemia major. In this article, a variety of clinical morbidities are explored, and their associations with the underlying disease pathophysiology and risk factors are examined. These involve several organs and organ systems including the vasculature, heart, liver, endocrine glands, bone, and the extramedullary hematopoietic system. The effects of some therapeutic interventions on the development of clinical complications are also discussed.

Address: Department of Medicine and Medical Specialties, IRCCS Cà Granda Foundation Maggiore Policlinico Hospital, 20122 Milan, Italy.

DOI: 10.1101/cshperspect.a013482

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MeSH Terms: Anticoagulants, Blood Transfusion, Endocrine System Diseases, Hematopoiesis, Extramedullary, Humans, Hypertension, Pulmonary, Iron, Iron Chelating Agents, Iron Overload, Leg Ulcer, Risk Factors, Splenectomy, Thromboembolism, Thrombophilia, beta-Thalassemia