儿童肝炎相关再生障碍性贫血的临床特征

Clinical features of hepatitis-associated aplastic anemia in children

摘要:

目的：探讨儿童肝炎相关再生障碍性贫血(HAAA)临床特征。方法：回顾性地研究2007年1月至2008年12月该院诊治的HAAA患儿的临床资料，包括临床表现、血常规、骨髓象、病毒血清学、免疫功能指标及治疗和预后。结果：共收治HAAA患儿8例，占同期获得性再生障碍性贫血（再障）的4.9%；男7例、女1例；平均7.5岁（4.4～10.3岁）。肝炎病因不明。患肝炎后至血细胞减少的间隔时间中位数为6周。3例为重症再障（SAA），5例极重型再障（VSAA）。全部病例均有严重T细胞免疫紊乱，Ts细胞明显升高、Th细胞明显下降。4例免疫治疗有效，3例起病1个月内死亡, 1例放弃治疗。结论：儿童HAAA发病率男性高于女性，学龄期多见。HAAA儿童存在严重T细胞免疫紊乱，早期死亡率高，免疫抑制治疗有效。［中国当代儿科杂志，2010，12（8）：609-612］

Abstract:

OBJECTIVE: To study the clinical features of hepatitis-associated aplastic anemia (HAAA) in children. METHODS: The clinical data of the children with newly diagnosed HAAA from January 2007 to December 2008 were respectively studied, including clinical manifestations, and blood routine, bone marrow examination, viral serology and immune function results as well as treatment and prognosis. RESULTS: A total of 8 children were confirmed as HAAA, accounting for 4.9% in children with aplastic anemia. There were 7 males and 1 female. The median age was 7.5 years (range 4.4 to 10.3 years) at diagnosis. They had negative serologic results and the causes of hepatitis could not be identified. The median interval from hepatitis occurrence to blood cell reduction was 6 weeks. Three cases were diagnosed as severe aplastic anemia and 5 cases as very severe aplastic anemia. Severe T cell immune disorders were found in all 8 cases. The percentage of Ts cells increased and the percentage of Th cells decreased significantly in the 8 children with HAAA. Four children survived after immune suppress treatment, three children died within one month after diagnosis and one child required own discharge without treatment. CONCLUSIONS: HAAA is more frequent in male school children. The children with HAAA have severe T cell immune disorders, with a higher early death rate. Immune suppress treatment is effective.［Chin J Contemp Pediatr, 2010, 12 (8):609-612］