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目的探讨系统性红斑狼疮(SLE)血液学异常的临床特点。方法对1992年2月-2010年12月在本科住院的首次确诊为SLE的26例患儿进行回顾性分析。男3例,女23例,男女比例为18;发病年龄4～18岁,平均13岁。根据1982年美国风湿病协会修订的SLE诊断标准及血液学异常的诊断标准,对其外周血常规、骨髓细胞形态、免疫学检查进行分析。结果 26例患儿中24例(92.3%)出现血液学异常,Hb、WBC、淋巴细胞及PLT减少分别为22例(84.6%)、8例(30.8%)、11例(45.8%)及15例(57.7%),其中一系减少6例(25.0%),两系减少15例(62.5%),三系减少3例(12.5%)。Coombs试验阳性12例(46.2%)。其中自身免疫性溶血性贫血10例(38.5%)。18例患儿进行了骨髓细胞学检查,骨髓增生活跃12例(66.7%),红系、粒系巨幼样改变各2例,红系核畸变3例,巨核细胞成熟障碍2例。26例患儿中误诊15例(61.5%)。结论血液系统是儿童SLE脏器损害最常见的靶器官之一,血液学异常可表现为一系或多系改变,骨髓形态学可有多种异常表现。对于初诊时有血液学异常的SLE患儿,应重视骨髓检查、免疫指标及抗核抗体等的检测,以减少误诊。
Objective To investigate the clinical features of hematological abnormalities in systemic lupus erythematosus (SLE). Methods A retrospective analysis was performed on 26 children admitted to undergraduate hospital for the first time from February 1992 to December 2010 with SLE. 3 males and 23 females, male to female ratio of 1 8; the age of onset of 4 to 18 years, mean 13 years. According to the SLE diagnostic criteria revised by the American College of Rheumatology in 1982 and the diagnostic criteria of hematological abnormalities, the peripheral blood, bone marrow cell morphology and immunological examination were analyzed. Results Twenty-four patients (92.3%) had hematological abnormalities. The reduction of Hb, WBC, lymphocytes and PLT were 22 cases (84.6%), 8 cases (30.8%), 11 cases (45.8%) and 15 Six cases (25.0%) were reduced by one system, 15 cases (62.5%) were reduced by two systems and three cases (12.5%) by three systems. Coombs test positive in 12 cases (46.2%). Including autoimmune hemolytic anemia in 10 cases (38.5%). Bone marrow cytology was performed in 18 patients. Twelve cases (66.7%) were active in bone marrow hyperplasia, two in erythroid and myeloid megaloblastic cells, three in erythroid and three in megakaryocyte. Twenty-six patients were misdiagnosed in 15 cases (61.5%). Conclusion The hematological system is one of the most common target organ damage in children with SLE. Hematological abnormalities may manifest as one or more changes in the system. There are many abnormalities in the morphology of the bone marrow. For newly diagnosed SLE children with hematologic abnormalities, bone marrow examination, immune index and anti-nuclear antibody should be taken to reduce misdiagnosis.