儿童肝炎相关再生障碍性贫血的诊治进展

Progress in diagnosis and treatment of hepatitis associated aplastic anemia in children

儿童肝炎相关再生障碍性贫血(hepatitis associated aplastic anemia, HAAA)简称肝炎相关性再障,其外周血细胞减少、骨髓造血衰竭常在急性黄疸型肝炎之后数月内突然发生,是罕见的血液病。确切的病原学至今仍不清楚,但 T 淋巴细胞介导的免疫损伤肝脏及骨髓造血干细胞的机制明确,造血干细胞移植治疗为首选方案。本文就儿童HAAA的流行病学、诊断标准、发病原因及治疗方案进展作一综述。以期改善我国重型/极重型儿童 HAAA 患者的预后。

Hepatitis associated aplastic anemia (HAAA) in children is a rare hematologic disease, which is characterized by the sudden decrease of peripheral blood cells and sudden onset of bone marrow hematopoietic failure within several months after acute icteric hepatitis occurs. The exact etiology is still unknown at present, but the mechanism of immune injury in the liver and bone marrow hematopoietic stem cells mediated by T lymphocytes is clear. Hematopoietic stem cell transplantation is the preferred option of treatment. This article reviews the epidemiology, diagnostic criteria, etiology and treatment protocols of HAAA in children, in order to improve the prognosis of HAAA in severe/very severe children in China.