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目的研究原发性干燥综合征(pSS)伴中重度血小板减少的临床特征。方法回顾性分析2009年6月至2011年6月中国医科大学附属第一医院风湿免疫科31例pSS伴中重度血小板减少患者(pSS组)的临床资料,并与34例免疫性血小板减少性紫癜患者(ITP组)的临床资料进行比较。结果 pSS组和ITP组患者均以皮肤出血为主要出血表现,但口腔出血者pSS组明显低于ITP组。血小板相关免疫球蛋白(PAIg)测定方面,pSS组PAIgM高于ITP组,pSS组血小板计数与PAIgG和PAIgM呈负相关。经甲泼尼龙联合静脉注射免疫球蛋白治疗后pSS组1、2周有效率分别为58.6%,72.4%。结论 pSS伴中重度血小板减少患者以皮肤出血为主要出血表现,合并其他部位出血不多见,抗血小板抗体可能是导致血小板减少的重要因素之一,近期有效率与ITP组相近。
Objective To study the clinical features of primary Sjogren’s syndrome (pSS) with moderate to severe thrombocytopenia. Methods The clinical data of 31 patients with pSS severe thrombocytopenia (pSS group) from the Department of Rheumatology, the First Affiliated Hospital of China Medical University from June 2009 to June 2011 were retrospectively analyzed. The clinical data of 34 patients with immune thrombocytopenic purpura The clinical data of patients (ITP group) were compared. Results The skin hemorrhage was the main bleeding in pSS group and ITP group, but the level of pSS in oral hemorrhage was significantly lower than that in ITP group. Platelet-associated immunoglobulin (PAIg) determination, pSS group PAIgM higher than the ITP group, pSS group platelet count and PAIgG and PAIgM was negatively correlated. The effective rate of pSS group was 58.6% and 72.4% respectively after the combination of methylprednisolone and intravenous immunoglobulin. Conclusion Bleeding is the main bleeding in pSS patients with moderate to severe thrombocytopenia. Bleeding in other parts is rare. Antiplatelet antibody may be one of the important factors leading to thrombocytopenia. The recent efficiency is similar to ITP group.