Early evidence of bone marrow dysfunction in children with indeterminate fulminant hepatic failure who ultimately develop aplastic anemia
Early evidence of bone marrow dysfunction in children with indeterminate fulminant hepatic failure who ultimately develop aplastic anemia
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DOI：
10.1111/j.1600-6143.2004.00559.x
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发表时间：
2004-10-01
影响因子：
8.8
通讯作者：
Martín, MG
中科院分区：
文献类型：
--
作者：
Molina, RA;Katzir, L;Martín, MG
在儿童中，再生障碍性贫血(AA)是与暴发性肝功能衰竭(FHF)相关的常见并发症。这项研究的目的是确定是否可以使用移植前特定的临床和实验室特征来区分患有FHF的患者，这些患者患再生障碍性贫血的风险更高。我们进行了一项回顾性病例对照研究，以评估那些有FHF证据并最终发展为再生障碍性贫血的患者的临床和实验室特征。我们确诊了9名AA患者，所有患者都患有不明形式的FHF，并接受了肝移植(LTX)。将AA患者与47名接受移植但未发生AA的不明原因FHF患者作为对照组进行比较。我们发现，在患再生障碍性贫血的患者中，男性比例过高(p=0.01)。此外，在移植前阶段，与对照组相比，AA组的白细胞计数(p=0.005)、绝对淋巴细胞计数(p=0.004)和血小板计数(p=0.019)显著降低。我们的结论是，在肝移植和部分不明形式的FHF患者发生再生障碍性贫血之前，早期骨髓功能障碍的证据是明显的。
In children, aplastic anemia (AA) is a common complication associated with fulminant hepatic failure (FHF). The objective of this study was to determine whether specific pretransplantation clinical and laboratory characteristics can be used to distinguish between patients with FHF who are at higher risk of developing AA. We performed a retrospective case-control study to evaluate the clinical and laboratory characteristics of those patients who presented with evidence of FHF and eventually developed aplastic anemia. We identified nine patients with AA, and all had the indeterminate form of FHF and underwent liver transplantation (LTx). The AA patients were compared with a control group of 47 patients with indeterminate FHF that underwent transplantation and did not develop AA. We found that males were over-represented in the group of patients that developed AA (p = 0.01). Furthermore, during the pretransplant period, the AA group had a significantly lower white count (p = 0.005), absolute lymphocyte count (p = 0.004), and platelet count (p = 0.019) when compared with controls. We conclude that evidence of early bone marrow dysfunction is apparent before liver transplantation and the development of AA in a subset of patients with the indeterminate form of FHF.