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原发性抗磷脂综合征和系统性红斑狼疮中的自身免疫性血小板减少症:脾切除术的疗效

Autoimmune thrombocytopenia in primary antiphospholipid syndrome and systemic lupus erythematosus: the response to splenectomy.

作者信息

Hakim A J, Machin S J, Isenberg D A

机构信息

Centre for Rheumatology, Department of Medicine, University College London, United Kingdom.

出版信息

Semin Arthritis Rheum. 1998 Aug;28(1):20-5. doi: 10.1016/s0049-0172(98)80024-3.

DOI:10.1016/s0049-0172(98)80024-3
PMID:9726332
Abstract

OBJECTIVE

To study the outcome of splenectomy in the management of thrombocytopenia.

METHODS

Cases of systemic lupus erythematosus (SLE) or primary antiphospholipid syndrome (PAPS) complicated by severe thrombocytopenia were identified from a database of patients attending outpatients over the period 1978 to 1996. Clinical presentation, laboratory investigations, and response to medical treatment and/or splenectomy were documented.

RESULTS

A total of 17 patients had severe thrombocytopenia; splenectomy was performed on 13: 9 with SLE, 3 with PAPS, and 1 with lupus-like disease (LLD). After splenectomy, six of nine patients with SLE and all three patients with PAPS gained complete remission of thrombocytopenia. There were no complications from splenectomy. The remaining four patients, three with SLE and one with PAPS, remained in a stable partial or full remission with oral medication and did not require splenectomy.

CONCLUSION

This study re-emphasizes the place for splenectomy in SLE patients and supports its role in the management of thrombocytopenia in PAPS.

摘要

目的

研究脾切除术治疗血小板减少症的效果。

方法

从1978年至1996年门诊患者数据库中识别出系统性红斑狼疮(SLE)或原发性抗磷脂综合征(PAPS)并发严重血小板减少症的病例。记录临床表现、实验室检查以及对药物治疗和/或脾切除术的反应。

结果

共有17例患者出现严重血小板减少症;13例接受了脾切除术:9例为SLE患者,3例为PAPS患者,1例为狼疮样疾病(LLD)患者。脾切除术后,9例SLE患者中的6例以及所有3例PAPS患者的血小板减少症完全缓解。脾切除术无并发症发生。其余4例患者,3例为SLE患者,1例为PAPS患者,通过口服药物维持稳定的部分或完全缓解,无需进行脾切除术。

结论

本研究再次强调了脾切除术在SLE患者中的地位,并支持其在PAPS患者血小板减少症治疗中的作用。

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