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两例系统性红斑狼疮伴难治性血小板减少症对静脉注射低剂量环磷酰胺治疗有反应。

Two cases of refractory thrombocytopenia in systemic lupus erythematosus that responded to intravenous low-dose cyclophosphamide.

机构信息

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

出版信息

J Korean Med Sci. 2013 Mar;28(3):472-5. doi: 10.3346/jkms.2013.28.3.472. Epub 2013 Mar 4.

Abstract

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/µL. Corticosteroid is the first choice of medication for inducing remission, and immunosuppressive agents can be added when thrombocytopenia is refractory to corticosteroid or recurs despite it. We presented two SLE patients with thrombocytopenia who successfully induced remission after intravenous administration of low-dose cyclophosphamide (CYC) (500 mg fixed dose, biweekly for 3 months), followed by azathioprine (AZA) or mycophenolate mofetil (MMF). Both patients developed severe thrombocytopenia in SLE that did not respond to pulsed methylprednisolone therapy, and started the intravenous low-dose CYC therapy. In case 1, the platelet count increased to 50,000/µL after the first CYC infusion, and remission was maintained with low dose prednisolone and AZA. The case 2 achieved remission after three cycles of CYC, and the remission continued with low dose prednisolone and MMF.

摘要

治疗系统性红斑狼疮(SLE)伴发的血小板减少症,一般考虑在当前有出血、严重瘀斑或血小板计数<50×10^9/L 时进行。诱导缓解时,糖皮质激素是首选药物,当糖皮质激素治疗无效或复发时,可以加用免疫抑制剂。我们报告了 2 例 SLE 伴发血小板减少症患者,他们在接受静脉注射低剂量环磷酰胺(500mg 固定剂量,每 2 周 1 次,共 3 个月)治疗后成功诱导缓解,之后分别使用硫唑嘌呤或霉酚酸酯。这 2 例患者的 SLE 均出现严重的血小板减少症,对大剂量甲泼尼龙冲击治疗无反应,开始接受静脉注射低剂量环磷酰胺治疗。第 1 例患者在第 1 次输注环磷酰胺后血小板计数增至 50×10^9/L,随后维持小剂量泼尼松龙和硫唑嘌呤治疗缓解。第 2 例患者在 3 个周期的环磷酰胺治疗后达到缓解,随后维持小剂量泼尼松龙和霉酚酸酯治疗缓解。

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