Wu Jun, Cheng Yifei, Zhang Leping
Department of Pediatrics, Medical School, Peking University, The Affiliated People's Hospital of Peking University, Peking, China.
Department of Pediatrics, Medical School, Peking University, The Affiliated People's Hospital of Peking University, Peking, China.
Leuk Res. 2015 Dec;39(12):1347-52. doi: 10.1016/j.leukres.2015.09.012. Epub 2015 Sep 21.
This retrospective single-center study assessed the incidence and clinical features of immune manifestations of refractory cytopenia of childhood (RCC) and childhood aplastic anemia (AA). We evaluated 72 children with RCC and 123 with AA between February 2008 and March 2013. RCC was associated with autoimmune disease in 4 children, including 1 case each with autoimmune hemolytic anemia, rheumatoid arthritis, systemic lupus erythematosus, and anaphylactoid purpura. No children with AA were diagnosed with autoimmune diseases. Immune abnormalities were common in both RCC and AA; the most significant reductions were in the relative numbers of CD3-CD56+ subsets found in RCC. Despite the many similar immunologic abnormalities in AA and RCC, the rate of autoimmune disease was significantly lower in childhood AA than RCC (p=0.008, χ2=6.976). The relative numbers of natural killer cells were significantly lower in RCC patients than AA patients. By month 6, there was no significant difference in autoimmune manifestations between RCC and AA in relation to the response to immunosuppressive therapy (p=0.907, χ2=0.014). The large overlap of analogous immunologic abnormalities indicates that RCC and childhood AA may share the same pathogenesis.
这项回顾性单中心研究评估了儿童难治性血细胞减少症（RCC）和儿童再生障碍性贫血（AA）免疫表现的发生率及临床特征。我们对2008年2月至2013年3月期间的72例RCC患儿和123例AA患儿进行了评估。4例RCC患儿合并自身免疫性疾病，其中自身免疫性溶血性贫血、类风湿关节炎、系统性红斑狼疮及过敏性紫癜各1例。无AA患儿被诊断为自身免疫性疾病。免疫异常在RCC和AA中均很常见；RCC中最显著减少的是CD3-CD56+亚群的相对数量。尽管AA和RCC存在许多相似的免疫异常，但儿童AA的自身免疫性疾病发生率显著低于RCC（p=0.008，χ2=6.976）。RCC患者的自然杀伤细胞相对数量显著低于AA患者。到第6个月时，RCC和AA在免疫抑制治疗反应方面的自身免疫表现无显著差异（p=0.907，χ2=0.014）。类似免疫异常的大量重叠表明RCC和儿童AA可能具有相同的发病机制。
