原发性干燥综合征合并血小板减少288例的临床分析2.docVIP

原发性干燥综合征合并血小板减少288例的临床分析 [摘要] 目的 探讨原发性干燥综合征(pSS) 患者血小板减少的临床特征及治疗效果。方法 回顾分析288例pSS患者的临床资料。结果 227例无血小板减少组（A组）口干、眼干、猖獗齿、关节痛、脾大发生率低于61例血小板减少组（B组），A组血沉值、抗SSB阳性率低于B组。17例血小板减少患者行骨髓穿刺，1例骨髓增生低下，16例骨髓增生活跃，15例巨核细胞成熟障碍，2例淋巴细胞增多。B组主要使用糖皮质激素及免疫抑制剂治疗，无效者予甲泼尼龙冲击、静脉丙种球蛋白治疗，1例患者使用利妥昔单抗，1例使用促血小板生成素。结论 伴血小板减少的pSS患者更易出现口干、眼干、猖獗齿、关节痛、脾大，且血沉值及抗SSB阳性率更高。治疗上主要使用糖皮质激素及免疫抑制剂，无效者可考虑激素冲击、利妥昔单抗等治疗。 [关键词] 原发性干燥综合征； 血小板减少；治疗 [Abstract] Objective To investigate the clinical feature and therapeutic effect of primary Sjǒgren syndrome (pSS) patients complicated with thrombocytopenia． Methods We did a retrospective analysis on the clinical profiles of 288 pSS patients. Results The incidence of xerostomia, xerophthalmia, rampant caries, arthralgia, splenomegaly in the non-thrombocytopenia group(group A, 277 patients) was lower than thrombocytopenia group(group B, 61 patients). Compared to group B, the erythrocyte sedimentation rate and anti-SSB positive rate were lower in group A. 17 thrombocytopenia patients were received bone marrow aspiration, 1 bone marrow hyperplasia patient with inactive proliferation, 16 bone marrow hyperplasia patients with active proliferation, 15 patients with megakaryocytic maturation disorder, 2 patients with lymphocytosis. Glucocorticoids and immunosuppressive agents were mainly used in the group B. Non-responders were given methylprednisolone pluse treatment and gamma globulin IV therapy. 1 patient took Rituximab and 1 patient used thrombopoietin. Conclusion: pSS patients with thrombocytopenia are more likely to present xerostomia, xerophthalmia, rampant caries, arthralgia and splenomegaly symptoms as well as higher erythrocyte sedimentation rate and anti-SSB positive rate. Main drugs included glucocorticoids and immunosuppressive agents. Non-responders could be considered to receive methylprednisolone pluse treatment and Rituximab treatment. [Key words] primary Sjǒgren syndrome ；thrombocytopenia；therapeutic effect 干燥综合征（Sjǒgren syndrome，SS）是一种自身免