摘要
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 explore the clinical features of hematologic abnormalities in pediatric systemic lupus erythematosus（SLE）. Methods Twenty-six patients hospitalized in the Pediatric Department of People′s Hospital,Peking University from Feb.1992 to Dec.2010 were analyzed retrospectively.They were diagnosed as SLE for the first time.There were 3 boys and 23 girls,and boy to girl ratio was 18.The patients aged from 4 to 18 years old,with an average age of 13 years old.Complete blood count,bone marrow cell morphology,immunolo-gical tests were analyzed,according to the revised American College of Rheumatology classification criteria for SLE in 1982 and diagnostic criteria for hematologic abnormalities. Results Among the 26 children,24 children（92.3%） had hematologic abnormalities.The abnormal results of complete blood count included anemia in 22 cases（84.6%）,leukopenia in 8 cases（30.8%）,lymphopenia in 11 cases（45.8%） and thrombocytopenia in 15 cases（57.7%）.Single of the above hematologic abnormalities was involved in 6 cases（25.0%）,while bicytopenia in 15 cases（62.5%）and pancytopenia in 3 cases（12.5%）,respectively.There were 12 patients for positive Coombs test,and of them 10 patients were diagnosed as autoimmune hemolytic anemia.Bone marrow examination was carried out in 18 patients.Bone marrow hyperplasia was active in 12 patients（66.7%）.Megaloblastic changes in erythroid lineage were found in 2 patients,and the same as in myeloid lineage.Nuc-lear aberrations in erythroid lineage were found in 3 patients and megakaryocyte maturation disorders were found in 2 patients.Fifteen patients（61.5%） were misdiagnosed of the total 26 patients. Conclusions Hematological system is one of the most common target organs in pediatric SLE.Hematologic abnormalities include anemia,leukopenia,lymphopenia,thrombocytopenia,or bicytopenia and pancytopenia.There is a variety of morphological abnormalities of bone marrow.When those patients with hematologic abnormalities are first diagnosed,great importance should be attached on bone marrow examination,immunological tests,detection of antinuclear antibodies in order to reduce misdiagnosis.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2012年第21期1641-1643,共3页
Journal of Applied Clinical Pediatrics
关键词
系统性红斑狼疮
血液学
临床特点
儿童
systemic lupus erythematosus
hematology
clinical characteristics
child
