原发性干燥综合征 ( primarySj gren′ssyndrome,pSS)是好发于中青年女性的自身免疫病 ,虽主要累及外分泌腺体 ,但也可侵犯其他多个系统 ,血液系统累及可引起白细胞下降和血小板减少 ,但以自身免疫性溶血性贫血 (AIHA)为首发表现 ,无口眼干燥症状 ,青年发病的pSS临床上罕见。现 Primary Sjogren’s Syndrome (pSS) is an autoimmune disease that occurs in young and middle-aged women. Although it mainly affects exocrine glands, it can also invade other multiple systems. The blood system is involved in the pathogenesis of leukopenia and Thrombocytopenia, but with autoimmune hemolytic anemia (AIHA) as the first performance, no dry mouth symptoms, pSS clinical onset of young rare. Now