原发性干燥综合征伴血液系统损害的临床分析

目的 观察原发性干燥综合征(pSS)血液系统损害的特征.方法 回顾性分析49例pSS患者的临床资料,并对外周血和骨髓的改变进行分析.结果 49例患者中出现血液系统改变的有25例(51%),其中贫血15例(31%),白细胞减少8例(16%),血小板减少10例(20%).同时两系受累6例,3系均受累2例.23例行骨髓穿刺检查,均为骨髓增生明显活跃或增生正常,3系细胞形态正常;15例贫血者,14例骨髓铁染色强阳性或阳性;10例血小板减少者,骨髓检查示巨核细胞成熟障碍7例.结论 pSS并发血液系统损害多见,以贫血最为常见,并与铁代谢紊乱导致的骨髓红系造血细胞功能异常有关,而血小板减少与骨髓巨核细胞产板功能障碍有关.

Clinical Analysis of primary Sjogren Syndrome with Hematological damages

Objective To explore the characteristics of hematological changes in patients with primary Sj(o)gren'syndrome (pSS).Methods The clinical data of 49 cases with pSS were retrospectively analyzed.Resuits The data showed that 25 patients (51%) among 49 cases had hematological changes.of which 15 cases were anaemia (31%).8(16%) were leukopenia,and 10 cases (20%) were thrombocytopenia.And 6 cases had two cell lines involved.2 cases had three cell lines involved.Twenty-three bone marrow aspirations revealed active proliferation or normal cell proliferation and the morphology of all 3 cells lines were normal.Bone marrow examination had strong positive iton stain and 15 cases with anaemia showed positive iron stain.Seven out of 10 patients with thrombocytopenia had megakaryocyte maturation defect.Conclusion Hemotologieal changes are common.and anemia is the most common findings.Cytopenia is related to autoantibodies mediated stem cell damages.but anemia iS caused by iron metabolic disturbance.while thrombocytopenia is generally due to functional impairment of megakaryocytes.