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Semin Thromb Hemost 2003; 29(6): 595-604
DOI: 10.1055/s-2004-815627
Copyright © 2003 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Severe Chronic Idiopathic Thrombocytopenic Purpura during Childhood: Definition, Management, and Prognosis

George R. Buchanan1,2 , Janna M. Journeycake2 , Leah Adix2
  • 1Professor of Pediatrics, Director of Pediatric Hematology-Oncology, Division of Hematology-Oncology, Department of Pediatrics, The University of Texas Southwestern Medical Center at Dallas and The Center for Cancer and Blood Disorders, Children's Medical Center of Dallas, Dallas, Texas
  • 2Division of Hematology-Oncology, Department of Pediatrics, The University of Texas Southwestern Medical Center at Dallas and The Center for Cancer and Blood Disorders, Children's Medical Center of Dallas, Dallas, Texas
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Publication History

Publication Date:
13 January 2004 (online)

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ABSTRACT

Chronic idiopathic thrombocytopenic purpura (ITP) can be categorized as mild, moderately severe, or severe. Severe chronic ITP during childhood is a rare disorder characterized by clinically significant mucocutaneous hemorrhage, usually in the setting of marked thrombocytopenia. It can cause substantial morbidity and rarely mortality. Many patients improve with time or even fully recover, but for those whose quality of life is negatively influenced by hemorrhage or side effects of conventional therapy (corticosteroids, intravenous immunoglobulin G, or anti-D), splenectomy is recommended. If splenectomy is unsuccessful or not feasible, other drug treatments are available, but few efficacy data exist, and the toxicity and cost of these treatments can be appreciable. Their use is best avoided outside of clinical trials conducted in specialty centers or in multi-institutional networks.

KEYWORDS

ITP - thrombocytopenia - chronic - severe - splenectomy

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