单倍体外周血干细胞移植成功治疗急性肝炎合并肝真菌脓肿后严重再生障碍性贫血1例

K. Adachi, F. Tajima, Yuzuru Hosoda, H. Mochida, S. Sugihara, T. Okamoto, T. Kawatani, J. Suzumiya
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摘要

再生障碍性贫血(AA)以骨髓功能衰竭和骨髓各元素明显减少为特征。肝炎相关性AA(HAAA)最常发生在急性肝炎发作后两到三个月出现严重全血细胞减少的青少年和年轻男性。骨髓衰竭可能是危险和严重的,如果不治疗通常是致命的。此外,这种肝炎在临床上与典型的病毒性肝炎无法区分,但没有明确的病因。众所周知,如果对免疫治疗无反应和/或进展为严重全血细胞减少症,AA是一种潜在的致命疾病。尤其是侵袭性真菌感染(IFI),据报道预后非常差。虽然造血干细胞移植(HSCT)在这些AA患者中的作用正在发展,但即使现在也有一些患者与HSCT相关死亡。然而,考虑到严重感染的风险,建议早期同种异体造血干细胞移植(alloo -HSCT)应该在年轻的严重AA患者中进行。缺乏匹配的兄弟姐妹或无血缘关系的供体且有严重病例报告的AA患者
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Successful treatment of severe aplastic anemia following acute hepatitis with liver fungal abscess by haploidentical peripheral blood stem cell transplantation: a case report
Aplastic anemia(AA)is characterized by bone marrow failure and marked decreases of all marrow elements. Hepatitis-associated AA(HAAA)most often affects adolescent and young men who present with severe pancytopenia two to three months after an episode of acute hepatitis. The marrow failure can be precipitous and severe, and is usually fatal if untreated. Furthermore, the hepatitis can be clinically undistinguishable from typical viral hepatitis, but with no specific cause identified. It is well-known that AA is a potentially fatal condition if there is no response to immunotherapy and/or if there is progression to severe pancytopenia. In particular, invasive fungal infections(IFI)are reported to have very poor prognoses. While the role of hematopoietic stem cell transplantation (HSCT)in these AA patients is evolving, even now there are some patients with HSCT-related mortality. However, it is recommended that early allogeneic HSCT(allo-HSCT)should be done in younger severe AA patients with concerns about the risk of severe infections. Patients with AA who lack a matched sibling or unrelated donor and who have a severe Case report
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