以血栓性血小板减少性紫癜为首发表现的干燥综合征1例报告及文献复习

Thromobotic Thrombocytopenic Purpura as Initial Presentation of Primary Sjgren's Syndrome and Literatures Review

目的提高风湿科医生对干燥综合征合并血栓性血小板减少性紫癜的认识。方法报告一例干燥综合征合并血栓性血小板减少性紫癜病例并复习相关文献。结果患者为41岁女性,急性起病,以头昏、乏力、发热伴晕厥为主要临床表现,免疫指标抗核抗体、抗SSA、抗SSB、抗RNP抗体阳性,多次查血象三系均降低。血涂片发现有红细胞碎片。唾液腺检查发现异常,眼科检查发现干眼症。经血浆置换、输住新鲜冰冻血浆、糖皮质激素及免疫抑制剂长春新碱和环磷酰胺等治疗后病情好转。结论与干燥综合征相关的TTP鲜见报道,其发病率低,死亡率高,如诊断及治疗不及时,将严重威胁病人的生命。因其发病机制、临床表现及治疗方面较复杂,应于重视。

Objective： To improve the rheumatologists＇ recognition of thrombotic thrombocytopenie purpura complicating with Sjogren＇s syndrome. Methods： A case of thrombotic thrombocytopenic purpura with Sjogren＇s syndrome was reported and the related literatures were reviewed. Result： A previously healthy 41 - year - old woman presented with an acute onset of marked dizzy, fatigue and the appearance of fever and faint. Peripheral blood smear was notable for thrombecytopenia as well as anisocytosi, poikilocytosis and numerous fragmented red blood cells. Schirmer＇ s test was bilaterally positive, associated with positive Rose Bengal corneal staining. Glands radionuclide imaging revealed abnormal. The appearance of anti - SSA antibody and anti - SSB antibody confirmed the diagonosis of SS. The patient was treated sucessfully by plasma exchange therapy, plasma infusion and the use of glucocorticoid and immunodepressant. Her condition remained stable after haft a month. Conclusion： Thrombotic thrombocytopenic purpura complicating with Sjogren＇s syndrome has been described only a few times, Rheumatologists should pay attention to in the differention diagnosis because the diasease is rare and has high mortality.