Hepatitis-associated Aplastic Anaemia in Children
Hepatitis-associated Aplastic Anaemia in Children
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DOI：
10.1055/a-1108-1553
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发表时间：
2020-05-01
影响因子：
1
通讯作者：
Pfister, Eva-Doreen
中科院分区：
文献类型：
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作者：
Boeske, Anne-Kathrin;Sander, Annette;Pfister, Eva-Doreen
背景：特发性急性肝衰竭（IALF）患儿发展为危及生命的骨髓衰竭（BMF）的风险很高。该研究的目的是描述这种肝炎相关再生障碍性贫血（HAAA）的发展、治疗和预后，并与孤立的获得性再生障碍性贫血进行比较。结果回顾性分析1984 ~ 2017年18例HAAA患者，其中女性9例，年龄1.4 ~ 16.4岁。其中15例符合SAA标准，3例骨髓发育不全。其中11名儿童接受了肝移植（LTx）（42名因IALF接受肝脏移植的儿童中有11名（26%）接受了肝脏移植），6名患者未接受肝脏移植康复。在所有病例中，BMF、血小板减少和白细胞减少的最初症状都发生在LTx之前。随访期间，8例患者血液学缓解，6例接受造血干细胞移植。在首次出现症状后的平均304天内，有7名儿童死亡，主要原因是出血和感染。迄今为止，广泛的调查未能发现遗传、病毒或免疫病因。在同一时期接受肝移植的41例已知原因的ALF患者中，未诊断出AA。作为对照组，我们收集了孤立性SAA患者的资料。73%的患者在不进行HSCT的情况下接受免疫抑制治疗（IST）后获得缓解，随访期间无患者死亡。结论IALF患者应及早、定期（发病后0 ~ 22天）检查血球计数。积极的LTx、IST和HSCT治疗似乎可以改善预后。
Background Children with idiopathic acute liver failure (IALF) are at a high risk of developing life-threatening bone marrow failure (BMF). The aim of the study was to describe the development, therapy and prognosis of this hepatitis-associated aplastic anaemia (HAAA) in comparison to isolated acquired aplastic anaemia.Results We retrospectively found 18 patients (9 female) of HAAA between 1984 and 2017 with an age of 1.4-16.4 years. Fifteen of them fulfilled the SAA criteria, 3 had a bone marrow hypoplasia. Eleven of these children received liver transplantation (LTx) (these were 11 of 42 (26%) children receiving LTx for IALF), 6 patients recovered without LTx. The first signs of BMF, thrombocytopaenia and leucocytopaenia, occurred before LTx in all cases. During the follow-up period 8 patients reached haematological remission, 6 received haematopoietic stem cell transplantation (HSCT). Seven children died in a median of 304 days after the first symptoms mostly because of bleedings and infections. To date, extensive investigations failed to detect a genetically, viral or immunological aetiology. No AA was diagnosed in the 41 patients receiving liver transplants during the same period for ALF of known aetiology. As a comparison group, we collected the data of patients with isolated SAA. 73% achieved a remission after Immunosuppressive therapy (IST) without HSCT, and none of them died during the follow-up period.Conclusion Blood counts should be examined early and regularly (0-22 days after onset) in patients with IALF. Aggressive treatment with LTx, IST and HSCT appears to improve the prognosis.