巨核细胞增殖与分化障碍在系统性红斑狼疮伴发血小板减少中的作用

The role of megakaryocyte proliferation and differentiation in systemic lupus erythematosus patients with thrombocytopenia

目的分析巨核细胞在系统性红斑狼疮(SLE)伴血小板减少中的作用,探讨SLE血小板减少的发病机制。方法根据血小板减少相关实验室指标和骨髓检查结果对176例SLE伴发血小板减少的患者进行分析,并与32例血小板正常的SLE患者对比分析。结果176例SLE患者中有64例(36.4%)伴发血小板减少,其中血小板轻度减少48例(75%),重度减少16例(25%)。与32例血小板正常的SLE患者对照,伴血小板减少的SLE患者浆膜炎发生率高,肾脏损害程度较重,狼疮病情活动程度更高,补体C3水平在血小板减少组明显低于血小板正常组(P<0.05)。SLE伴血小板减少者骨髓巨核细胞数量较血小板正常的SLE患者明显减少(P=0.001),但其中幼稚巨核计数较血小板正常组增多(P=0.019),而产板巨核细胞计数较血小板正常组患者明显减少(P=0.000)。结论SLE伴发血小板减少多见于活动性病情严重的患者,其巨核细胞数量减少,分化中以幼稚巨核细胞为主,产板型巨核细胞则明显减少,提示巨核祖细胞增殖分化成熟障碍与SLE伴发血小板减少有关。

Objective To investigate the function of megakaryocytic cells in systemic lupus erythematosus（SLE）patients with thrombocytopenia.Methods Standard hematological tests,bone marrow examinations and immunological tests were carried out to scan thrombocytopenia in a total of 176 consecutive SLE patients,the clinical and laboratory data were compared between SLE patients with or without thrombocytopenia,and bone marrow examinations for analysis of megakaryocytic function in SLE patients with thrombocytopenia were compared with that in 32 SLE patients without thrombocytopenia.Results Thrombocytopenia was identified in 64 SLE patients（36.4%）,among whom,48（75%）with mild thrombocytopenia and 16（25%）with severe thrombocytopenia.In comparison with patients without thrombocytopenia,SLE patients with thrombocytopenia had higher incidence of serositis,more sever renal damage,lower serum C3 levels（P〈0.05）,and higher disease activity scores（P〈0.05）.The total numbers of megakaryocytes was decreased in SLE patients with thrombocytopenia in comparision with that in SLE patients without thrombocytopenia（P=0.001）,especially the platelete-forming megakaryocyte.Whereas,the numbers of promegakaryocytes was markedly increased in SLE patients with thrombocytopenia（P=0.019）.Conclusions Thrombocytopenia in SLE is more likely developed in patients with active and severe disease.Impaired function in megakaryocytic proliferation and differentiation may contribute to the thrombocytopenia occurred in SLE patients.