系统性红斑狼疮伴血液系统损害的临床研究

Systemic lupus erythematosus complicating with hematological disorders

目的观察系统性红斑狼疮(SLE)血液系统损害的特征,探讨其临床意义。方法 145例确诊的SLE患者被纳入研究,分为贫血组、免疫性血小板减少性紫癜(ITP)组、白细胞减少组、对照组(无血液系统改变的SLE)四组。所有患者均接受常规血液学及免疫学检查,部分患者行骨髓检查。结果 113例(77.9%)SLE患者有血液学异常,贫血88例(60.7%),贫血的主要类型有慢性疾病性贫血(ACD)37例(42.0%)、缺铁性贫血(IDA)29例(33.0%)、自身免疫性溶血性贫血(AIHA)15例(17.0%)、其他7例(8.0%);白细胞减少46例(31.7%),<3.0×109/L14例;血小板减少57例(39.3%);无血液学异常32例(22.1%)。SLE并发AIHA的肾损伤发生率及SLE疾病活动指数(SLEDAI)较对照组高(P<0.05);AIHA组dsDNA、AnuA抗体、抗心磷脂抗体(ACL)的阳性率较对照组高(P<0.05),而C3明显降低(P<0.05)。42例患者行骨髓检查,36例增生活跃,6例增生低下;5例有骨髓细胞形态异常。结论 SLE并发血液系统损害多见,以贫血最为常见,其可能不仅与ACL等抗体诱导的外周成熟红细胞破坏有关,还与铁代谢紊乱导致的骨髓红系造血细胞功能异常有关;AIHA可能是SLE出现肾损害的危险因素,并可作为疾病活动的评判指标;血小板减少与巨核细胞成熟障碍有关。骨髓造血细胞受累亦可能是SLE并发血细胞损害的原因。

Objective To study the characteristics of hematological abnormalities in patients with systemic lupus erythematosus （SLE） and their associations with immunological and clinical parameters.Methods One hundred and forty-five consecutive SLE patients were enrolled into the study.The patients were divided into 4 groups：anemia group,low white blood cell group,immune thrombopenic purpura group and control group （SLE patients without hematological abnormalities）.Standard hematological and immunological tests were performed in all participants and the examination of bone marrow was performed in 42 patients.Results 77.9% of pSS patients had hematological abnormalities.The prevalence of leucocytopenia,anemia and thrombocytopenia were 31.7% （46 /145）,60.7% （88 /145） and 39.3% （57 /145） respectively.SLE patients with anemia included ACD 37 （42%）,IDA 29 （33%）,AIHA 15 （17%） and other reasons 7 （8%）.The prevalence of renal involvement was much higher in SLE patients with AIHA or WBC 〈 3.0 × 109 /L than that in control group （P 〈 0.05）,respectively.SLE patients with AIHA presented anti-dsDNA,AnuA,ACL antibodies increased while level of C3 reduced compared with control group （P 〈 0.05,respectively）.Abnormal bone marrow changes were observed in 5 SLE patients with morphological changes.Conclusions Hematological abnormalities are common in SLE patients,of which anemia is the most common findings.Cytopenia might be related to not only the damages of mature cell mediated by autoantibodies （such as ACL etc）,but also impaired red cells production because of iron metabolic disturbance;SLE with AIHA may be a risk factor of renal involvement,and thrombocytopenia was related to the functional impairment of megakaryocyte in producing plate.Bone marrow involvement may be another reason of SLE patients with hematological disorders.