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目的总结以血液系统异常为首诊的系统性红斑狼疮(SLE)的临床特征,以提高临床诊断水平。方法回顾分析416例血液系统异常患者的临床表现、外周血分析、骨髓细胞学、尿常规、血清自身抗体(ANA)、抗双链DNA抗体(A-dsDNA)、球蛋白测定及补体C3的检测结果。结果 416例血液系统异常患者中,符合美国风湿病学会1997年修订的SLE诊断标准的56例(占13.5%)。其中有贫血表现的40例,白细胞减少34例,血小板减少32例。血象一系减少8例,二系减少38例,全血细胞减少10例。早期误诊为自身免疫性溶血性贫血(AIHA)6例,原发性血小板减少性紫癜(ITP)8例,骨髓增生异常综合征(MDS)4例。结论 SLE以血液系统异常为首发临床表现多见。系统及周密的诊断思路,相关的实验室检查,结合临床具体分析及追踪随访,是减少漏诊、误诊的关键。
Objective To summarize the clinical features of systemic lupus erythematosus (SLE) with hematologic abnormalities as the first diagnosis to improve the clinical diagnosis. Methods The clinical manifestations, peripheral blood analysis, myelocytology, urine routine, serum autoantibodies (ANA), anti-double-stranded DNA antibody (A-dsDNA), globulin and complement C3 were analyzed retrospectively in 416 patients with abnormal blood system. result. Results Among 416 patients with abnormal blood system, 56 patients (13.5%) met the SLE diagnostic criteria revised by the American College of Rheumatology in 1997. There are 40 cases of anemia, leukopenia in 34 cases, thrombocytopenia in 32 cases. Blood decreased a series of 8 cases, 38 cases of secondary reduction, 10 cases of pancytopenia. Early misdiagnosed as autoimmune hemolytic anemia (AIHA) in 6 cases, 8 cases of idiopathic thrombocytopenic purpura (ITP), 4 cases of myelodysplastic syndrome (MDS). Conclusion SLE with abnormal blood system as the first clinical manifestations more common. System and careful diagnosis ideas, related laboratory tests, combined with specific clinical analysis and follow-up is to reduce the missed diagnosis, misdiagnosis of the key.