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Semin Thromb Hemost 2005; 31(6): 717-730
DOI: 10.1055/s-2005-925478
Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome in Children and Adolescents

Eric J. Lowe1 , Eric J. Werner1 , 2
  • 1Division of Pediatric Hematology-Oncology, Eastern Virginia Medical School, Children's Hospital of the King's Daughters, Norfolk, Virginia
  • 2Professor, Eastern Virginia Medical School, Children's Hospital of the King's Daughters, Norfolk, Virginia
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Publication History

Publication Date:
30 December 2005 (online)

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ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are both uncommon disorders that present with a microangiopathic hemolytic anemia and thrombocytopenia. Although for TTP, neurologic manifestations predominate, and in HUS renal dysfunction is virtually always present, there is significant overlap in their clinical presentation. In recent years, tremendous progress has been made in our understanding of the pathogenesis of these disorders. It is now apparent that there are subcategories of both TTP and HUS that can differ in their clinical manifestations, etiology, and management. For instance, although most cases of HUS are due to infection with organisms that produce Shiga-like toxin, other cases may be caused by defined genetic abnormalities. Similarly, TTP is usually caused by genetic or acquired deficiency of the ADAMTS13 enzyme; however, in other cases the relationship with this enzyme remains to be established. Management includes supportive care, plasma transfusions for genetic protein deficiencies, and plasma exchange transfusion for autoimmune TTP.

KEYWORDS

Thrombotic thrombocytopenic purpura (TTP) - hemolytic uremic syndrome (HUS) - microangiopathy - children

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Eric J WernerM.D. 

Professor, Children's Cancer and Blood Disorders Center, Children's Hospital of the King's Daughters, 601 Children's Lane, Norfolk, VA 23507

Email: eric.werner@chkd.org

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