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特发性血小板减少性紫癜:儿童和成人发病机制、诊断及管理的当前问题

Idiopathic thrombocytopenic purpura: current issues for pathogenesis, diagnosis, and management in children and adults.

作者信息

George James N

机构信息

Hematology/Oncology Section, The University of Oklahoma Health Sciences Center, PO Box 26901, Oklahoma City, OK 73190, USA.

出版信息

Curr Hematol Rep. 2003 Sep;2(5):381-7.

PMID:12932309
Abstract

Current issues for idiopathic thrombocytopenic purpura (ITP) in children and adults are reviewed. Appropriate initial management of ITP in children is controversial. Some physicians favor specific treatment, whereas other physicians advocate only supportive care until the expected spontaneous remission occurs. In adults, ITP is typically persistent. Treatment is appropriate only for severe and symptomatic thrombocytopenia because prevention of bleeding is the only goal. If patients do not respond to initial glucocorticoid treatment, splenectomy is the appropriate second modality, resulting in sustained remissions in more than 66% of patients. For patients who have severe and symptomatic thrombocytopenia after splenectomy, treatment options are unclear. Intensive immunosuppression may be effective, but stimulation of platelet production by thrombopoietin may also be effective. Because major bleeding in ITP is rare, physicians must be certain that the adverse effects of treatment are not worse than the risks of bleeding caused by ITP.

摘要

本文综述了儿童和成人特发性血小板减少性紫癜(ITP)的当前问题。儿童ITP的适当初始管理存在争议。一些医生倾向于特异性治疗,而其他医生则主张仅进行支持性护理,直至预期的自发缓解发生。在成人中,ITP通常是持续性的。仅在严重且有症状的血小板减少症时才适合进行治疗,因为预防出血是唯一目标。如果患者对初始糖皮质激素治疗无反应,脾切除术是合适的第二种治疗方式,超过66%的患者可实现持续缓解。对于脾切除术后出现严重且有症状的血小板减少症的患者,治疗选择尚不清楚。强化免疫抑制可能有效,但血小板生成素刺激血小板生成也可能有效。由于ITP中的大出血很少见,医生必须确定治疗的不良反应不超过ITP引起的出血风险。

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