The ITP syndrome: pathogenic and clinical diversity

Author:

Cines Douglas B.12,Bussel James B.3,Liebman Howard A.4,Luning Prak Eline T.1

Affiliation:

1. Departments of Pathology and Laboratory Medicine and

2. Medicine, University of Pennsylvania, Philadelphia;

3. Departments of Pediatrics, Obstetrics-Gynecology, and Medicine, Weill Medical Center College of Cornell University, New York, NY; and

4. Department of Medicine, University of Southern California, Los Angeles

Abstract

Abstract Immune thrombocytopenia (ITP) is mediated by platelet autoantibodies that accelerate platelet destruction and inhibit their production. Most cases are considered idiopathic, whereas others are secondary to coexisting conditions. Insights from secondary forms suggest that the proclivity to develop platelet-reactive antibodies arises through diverse mechanisms. Variability in natural history and response to therapy suggests that primary ITP is also heterogeneous. Certain cases may be secondary to persistent, sometimes inapparent, infections, accompanied by coexisting antibodies that influence outcome. Alternatively, underlying immune deficiencies may emerge. In addition, environmental and genetic factors may impact platelet turnover, propensity to bleed, and response to ITP-directed therapy. We review the pathophysiology of several common secondary forms of ITP. We suggest that primary ITP is also best thought of as an autoimmune syndrome. Better understanding of pathogenesis and tolerance checkpoint defects leading to autoantibody formation may facilitate patient-specific approaches to diagnosis and management.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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