THE THROMBOCYTOPENIC PURPURAS - RECOGNITION AND MANAGEMENT

Authors
Citation
S. Gillis, THE THROMBOCYTOPENIC PURPURAS - RECOGNITION AND MANAGEMENT, Drugs, 51(6), 1996, pp. 942-953
Citations number
104
Categorie Soggetti
Pharmacology & Pharmacy",Toxicology
Journal title
DrugsACNP
ISSN journal
00126667
Volume
51
Issue
6
Year of publication
1996
Pages
942 - 953
Database
ISI
SICI code
0012-6667(1996)51:6<942:TTP-RA>2.0.ZU;2-A
Abstract
Idiopathic thrombocytopenic purpura (ITP) and thrombotic thrombocytope nic purpura (TTP), are distinct entities. ITP is a relatively common a utoimmune disorder typically manifesting with isolated thrombocytopeni a. The acute form, more common in children, is a self-limiting, often post-viral disease. Therapy, if indicated, usually consists of a brief course of steroids or intravenous IgG. Chronic ITP, more common in ad ults, rarely remits spontaneously. Most patients respond initially to steroids, but generally the disease relapses when steroids are tapered . Splenectomy offers a 70% chance of cure. A variety of treatment opti ons exist for patients not responding to splenectomy. The treating phy sician must choose the most effective and least toxic treatment for th e individual patient. TTP is a rare, often life-threatening, multisyst em disease of unknown aetiology. Its hallmark is widespread occlusion of the microcirculation by platelet aggregates. The clinical symptoms usually respond dramatically to plasma exchange therapy. Steroids, ant iplatelet agents and vincristine may also be useful. Splenectomy shoul d be considered in patients with multiple relapses. More specific ther apy awaits a fuller understanding of the pathogenesis of this disease.