摘要
目的总结系统性红斑狼疮(SLE)相关的再生障碍性贫血(AA)的临床特点,以提高对该病的认识。方法对收治的2例及文献报道的19例儿童SLE相关AA患者进行回顾性分析,并与单纯SLE及普通AA进行比较。结果 21例患者SLE和AA常不能同时诊断(占90.4%),SLE与AA的诊断相隔时间为-1～9年。实验室检查中抗ds-DNA抗体(42.8%)及补体降低(38.9%)的阳性率低于单纯SLE,外周血淋巴细胞比例升高(23.5%)及网织红细胞降低(17.6%)的发生率均低于普通AA,骨髓像、骨髓病理(100%)示骨髓造血不良,T细胞亚群中CD4+降低,CD8+升高,CD4/CD8倒置。治疗的有效率达85.7%,病死率约9.5%。结论 SLE相关AA临床表现无特异性;表现为外周血三系持续减少的患者应尽早作骨髓检查及免疫指标检测,以减少漏诊;其骨髓像、骨髓病理及T细胞亚群的特点与普通AA相似,但预后明显好于普通AA。
Objective To review the clinical features,pathogenesis,diagnosis,treatment,and prognosis of patients with systemic lupus erythematosus（SLE）-associated aplastic anemia（AA）,and to enhance the understanding of the disease.Methods Clinical data of two patients treated in Peking Union Medical College Hospital and 19 patients reported in the literature were retrospectively analyzed and comparisons among SLE associated AA and simple SLE or simple AA were made.Results SLE was diagnosed either before or after the diagnosis of AA in most cases（90.4%）.AA occurred one year before or 9 years after SLE diagnosis.Positive rate of anti-dsDNA antibody（42.8%） and decrease of C3 level（38.9%） were both less in SLE-associated AA patients than those with simple SLE.The increased percentage of lymphocytes in the peripheral blood（23.5%） and reduction of reticulocytes were both less in SLE-associated AA patients than those with simple AA.Hypocellularity was revealed by bone marrow aspiration and biopsy.T cell subgroups analysis showed that CD4-positive T cell was decreased,CD8-positive cells increased,and CD4/CD8 ratio was inverted.The overall response rate was 85.7%,and the mortality was 9.5% in SLE-associated AA.Conclusions Reports about SLE-associated AA are rare.Clinical manifestations of SLE-associated AA are not specific.Bone marrow examination and blood immunologiical parameters analysis should be done for patients with persistent pancytopenia to reduce misdiagnosis.The features of bone marrow and T cell subgroups in patients with SLE-associated AA were similar to those with simple AA,but the prognosis of the former was considerably better than that with simple AA.
出处
《临床儿科杂志》
CAS
CSCD
北大核心
2010年第6期560-564,共5页
Journal of Clinical Pediatrics
