Tung J, Hadzic N, Layton M, Baker A J, Dhawan A, Rela M, Heaton N D, Mieli-Vergani G
Department of Child Health, King's College Hospital, United Kingdom.
J Pediatr Gastroenterol Nutr. 2000 Nov;31(5):557-61. doi: 10.1097/00005176-200011000-00019.
Aplastic anemia is a rare but well-recognized complication of acute hepatitis and acute liver failure. The cause is unknown, and the condition is fatal without bone marrow recovery. Treatment includes immunosuppression regimens or bone marrow transplantation. The purpose of this study was to investigate the incidence, cause, treatment, and outcomes of this disorder in children.
Retrospective chart review of 75 patients with acute liver failure in a major pediatric liver center.
Eight patients had evidence of bone marrow failure. Of those, six had aplastic anemia, and two had transient bone marrow suppression. There were five boys, median age 57 months (range, 36-132 months). Two had parvovirus B19, six had non-A, non-B, non-C hepatitis. Five underwent liver transplantation: auxiliary in one, orthotopic in four. The interval between initial symptoms and development of aplastic anemia and/or bone marrow suppression was 21 to 99 days (median, 39 days). Four patients with aplastic anemia received intravenous antithymocyte globulin (ATG) or antilymphocyte globulin (ALG). Median recovery period of granulopoiesis was 62 days (range, 27-115 days). Two made a full recovery, one had myelodysplasia, and one with unresponsive disease died of septic complications. Four did not receive ATG/ALG, two had aplastic anemia, and two had bone marrow suppression. Three underwent liver transplantation, and all four resumed granulopoiesis. One child who underwent liver transplantation died of sepsis with chronic rejection. Median recovery of granulopoiesis was 99 days (range, 20-153 days).
Bone marrow failure occurs in 10.7% of children with acute liver failure. It sometimes occurs in association with non-A, non-B, non-C hepatitis and parvovirus B19 infection. Treatment with ATG/ALG is successful and is well tolerated in most cases.
再生障碍性贫血是急性肝炎和急性肝衰竭一种罕见但已被充分认识的并发症。病因不明，若骨髓未恢复，病情会致命。治疗方法包括免疫抑制方案或骨髓移植。本研究的目的是调查儿童中这种疾病的发病率、病因、治疗及转归。
对一家大型儿科肝脏中心75例急性肝衰竭患者进行回顾性病历审查。
8例患者有骨髓衰竭证据。其中，6例为再生障碍性贫血，2例为短暂性骨髓抑制。有5名男孩，中位年龄57个月（范围36 - 132个月）。2例感染细小病毒B19，6例为非甲、非乙、非丙型肝炎。5例接受了肝移植：1例为辅助性肝移植，4例为原位肝移植。从初始症状到再生障碍性贫血和/或骨髓抑制出现的间隔时间为21至99天（中位时间39天）。4例再生障碍性贫血患者接受了静脉注射抗胸腺细胞球蛋白（ATG）或抗淋巴细胞球蛋白（ALG）。粒细胞生成的中位恢复期为62天（范围27 - 115天）。2例完全康复，1例发生骨髓发育异常，1例病情无反应死于败血症并发症。4例未接受ATG/ALG，2例为再生障碍性贫血，2例为骨髓抑制。3例接受了肝移植，所有4例均恢复粒细胞生成。1例接受肝移植的儿童死于伴有慢性排斥反应的败血症。粒细胞生成的中位恢复期为99天（范围20 - 153天）。
急性肝衰竭儿童中10.7%会发生骨髓衰竭。有时与非甲、非乙、非丙型肝炎及细小病毒B19感染相关。ATG/ALG治疗成功，多数情况下耐受性良好。
