The thrombocytopenic purpuras - Recognition and management

被引:18
作者
Gillis, S
机构
[1] Division of Hematology-Oncology, New England Medical Center, Boston, MA
[2] Division Hematology-Oncology, New England Medical Center, Boston, MA 02111
关键词
D O I
10.2165/00003495-199651060-00003
中图分类号
R9 [药学];
学科分类号
1007 ;
摘要
Idiopathic thrombocytopenic purpura (ITP) and thrombotic thrombocytopenic purpura (TTP), are distinct entities. ITP is a relatively common autoimmune disorder typically manifesting with isolated thrombocytopenia. 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 adults, 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 options exist for patients not responding to splenectomy. The treating physician must choose the most effective and least toxic treatment for the individual patient. TTP is a rare, often life-threatening, multisystem 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, antiplatelet agents and vincristine may also be useful. Splenectomy should be considered in patients with multiple relapses. More specific therapy awaits a fuller understanding of the pathogenesis of this disease.
引用
收藏
页码:942 / 953
页数:12
相关论文
共 105 条
[1]   DANAZOL FOR THE TREATMENT OF IDIOPATHIC THROMBOCYTOPENIC PURPURA [J].
AHN, YS ;
HARRINGTON, WJ ;
SIMON, SR ;
MYLVAGANAM, R ;
PALL, LM ;
SO, AG .
NEW ENGLAND JOURNAL OF MEDICINE, 1983, 308 (23) :1396-1399
[2]   ACUTE IMMUNE THROMBOCYTOPENIC PURPURA - A COMPARATIVE-STUDY OF VERY HIGH ORAL DOSES OF METHYLPREDNISOLONE AND INTRAVENOUSLY ADMINISTERED IMMUNE GLOBULIN [J].
ALBAYRAK, D ;
ISLEK, I ;
KALAYCI, AG ;
GURSES, N .
JOURNAL OF PEDIATRICS, 1994, 125 (06) :1004-1007
[3]   THROMBOTIC THROMBOCYTOPENIC PURPURA - REPORT OF 16 CASES AND REVIEW OF LITERATURE [J].
AMOROSI, EL ;
ULTMANN, JE .
MEDICINE, 1966, 45 (02) :139-+
[4]   RESPONSE OF RESISTANT IDIOPATHIC THROMBOCYTOPENIC PURPURA TO PULSED HIGH-DOSE DEXAMETHASONE THERAPY [J].
ANDERSEN, JC .
NEW ENGLAND JOURNAL OF MEDICINE, 1994, 330 (22) :1560-1564
[5]  
Bachman WR, 1996, AM J HEMATOL, V51, P93, DOI 10.1002/(SICI)1096-8652(199601)51:1<93::AID-AJH16>3.0.CO
[6]  
2-C
[7]   URGENT TREATMENT OF IDIOPATHIC THROMBOCYTOPENIC PURPURA WITH SINGLE-DOSE GAMMA-GLOBULIN INFUSION FOLLOWED BY PLATELET TRANSFUSION [J].
BAUMANN, MA ;
MENITOVE, JE ;
ASTER, RH ;
ANDERSON, T .
ANNALS OF INTERNAL MEDICINE, 1986, 104 (06) :808-809
[8]   IMMUNOADSORPTION WITH PROTEIN-A SEPHAROSE OR SILICA [J].
BELAK, M ;
WIDDER, RA ;
BRUNNER, R ;
BORBERG, H ;
HAUPT, WF .
LANCET, 1994, 343 (8900) :792-793
[9]   IMPROVED SURVIVAL IN THROMBOTIC THROMBOCYTOPENIC PURPURA HEMOLYTIC UREMIC SYNDROME - CLINICAL-EXPERIENCE IN 108 PATIENTS [J].
BELL, WR ;
BRAINE, HG ;
NESS, PM ;
KICKLER, TS .
NEW ENGLAND JOURNAL OF MEDICINE, 1991, 325 (06) :398-403
[10]  
BELLUCCI S, 1988, BLOOD, V71, P1165