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自身免疫性血小板减少性紫癜

Autoimmune thrombocytopenic purpura.

作者信息

Bussel J B

机构信息

Division of Hematology-Oncology, Cornell University Medical Center, New York, New York.

出版信息

Hematol Oncol Clin North Am. 1990 Feb;4(1):179-91.

PMID:1690204
Abstract

The classic therapies of ITP are corticosteroids and splenectomy. These therapies will suffice to treat the majority of patients. In any patient with ITP in whom an urgent rise in the platelet count is required, especially children with acute ITP at diagnosis, gammaglobulin in combination with corticosteroids is very effective. In children with persistent ITP, avoiding splenectomy by using IVGG maintenance has been shown to be an effective form of therapy. For adults, most patients will be adequately treated by a short course of steroids followed by splenectomy, if needed. Other treatments are most needed in those adults whose ITP fails to respond to splenectomy or those who have special conditions. Danazol, vinca alkaloids, oral cyclophosphamide, and IVGG appear to be effective as chronic maintenance therapies, especially of splenectomized patients who are still thrombocytopenic. The role of newer therapies such as staph protein A pheresis and intravenous anti-D remains to be more completely explored.

摘要

特发性血小板减少性紫癜(ITP)的经典疗法是使用皮质类固醇和进行脾切除术。这些疗法足以治疗大多数患者。对于任何需要血小板计数紧急上升的ITP患者,尤其是诊断时患有急性ITP的儿童,γ球蛋白联合皮质类固醇非常有效。对于持续性ITP儿童,通过使用静脉注射免疫球蛋白(IVGG)维持治疗来避免脾切除术已被证明是一种有效的治疗方式。对于成年人,大多数患者通过短期使用类固醇治疗,必要时再进行脾切除术,即可得到充分治疗。其他治疗方法最适用于那些ITP对脾切除术无反应的成年人或有特殊情况的成年人。达那唑、长春花生物碱、口服环磷酰胺和IVGG似乎作为慢性维持疗法有效,特别是对于脾切除术后仍有血小板减少症的患者。诸如葡萄球菌蛋白A分离术和静脉注射抗-D等新疗法的作用仍有待更全面地探索。

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