原发性干燥综合征伴中重度血小板减少31例分析

Primary Sjgren's syndrome complicated with moderate-to-severe thrombocytopenia

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 investigate the clinical features of primary Sjogren＇s syndrome （pSS） complicated with moderate- to-severe thrombocytopenia. Methods We did a retrospective analysis on the clinical profiles of 31 patients with pSS complicated with moderate-to-severe thrombocytopenia （ group pSS） and 34 with immune thrombocytopenic purpura （ group ITP） who were admitted to the department of rheumatology and immunology,The First Affiliated Hospital of China Medical University,between June 2009 and June 2011, for comparison on the clinical features. Results Both groups were mainly featured by cutaneous hemorrhage. A lower incidence of oral hemorrhage was noted in group pSS, but not in group ITP. Of all platelet-associated immunoglobulins （PAIg）,the level of PAIgM in group ITP was lower than that in group pSS, in which the platelet count was negatively correlated with PAIgG and PAIgM. In group pSS, methylprednisolone and immunoglobulin intravenous administration yielded an effective rate of 58.6% and 72.4% at weeks 1 and 2,respectively. Conclusion Pa- tients with pSS and concomitant moderate-to-severe thrombocytopenia are mainly featured by cutaneous hemorrhage and infrequent incidence of hemorrhage at miscellaneous sites. Anti-platelet antibodies are probably the major contributor of pSS- associated thrombocytopenia. The short-term treatment effectiveness does not differ significantly between patients with pSS