Hematopoietic Stem Cell Transplantation for Familial Hemophagocytic Lymphohistiocytosis and Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in Japan
Hematopoietic Stem Cell Transplantation for Familial Hemophagocytic Lymphohistiocytosis and Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in Japan
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DOI：
10.1002/pbc.22310
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发表时间：
2010-02-01
影响因子：
3.2
通讯作者：
Hara, Toshiro
中科院分区：
文献类型：
--
作者：
Ohga, Shouichi;Kudo, Kazuko;Hara, Toshiro
背景。在日本对噬血细胞性淋巴组织细胞增多症 (HLH) 患者的移植后结果进行了分析，该国与 Epstein-Barr 病毒 (EBV) 相关的严重形式存在问题。方法。根据全国登记，纳入了 1995 年至 2005 年间接受干细胞移植 (SCT) 的 57 名患者（43 名家族性 HLH [12 名 FHL2、11 名 FHL3、20 名未定义]、14 名 EBV-HLH）。结果。 57 名患者接受了 61 次 SCT，其中包括 4 次连续 SCT。 93% 的病例采用同种异体供体进行 SCT（异体 53 例，双胞胎 1 例，自体 3 例）。半数病例采用了无关供体脐带血移植（UCBT）（21 例 FHL，7 例 EBV-HLH）。 26% 的病例使用了降低强度调节。 10 年总体生存率（平均 +/- SE%）为 65.0 +/- 7.9%。 FHL 和 85.7 +/- 9.4%。分别在 EBV-HLH 患者中。 UCBT 接受者的存活率 >65%。 FHL 和 EBV-HLH 患者均存在这种情况。第二次 UCBT 后，四分之三的患者成功植入。 FHL 患者由于早期治疗相关死亡而表现出较差的结果（
Background. Post-transplant outcomes of hemophagocytic lymphohistiocytosis (HLH) patients were analyzed in Japan where Epstein-Barr virus (EBV)-associated severe forms are problematic. Methods. Fifty-seven patients (43 familial HLH [12 FHL2, 11 FHL3, 20 undefined], 14 EBV-HLH) who Underwent Stem Cell transplantation (SCT) between 1995 and 2005 were enrolled based oil the nationwide registration. Results. Fifty-seven patients underwent 61 SCTs, including 4 consecutive SCTs. SCTs were employed using allogeneic donors in 93% of cases (allo 53, twin 1, auto 3). Unrelated donor cord blood transplantation (UCBT) was employed in half of cases (21 FHL, 7 EBV-HLH). Reduced intensity conditioning was used in 26% of cases. The 10-year overall Survival rates (rnedian +/- SE%) were 65.0 +/- 7.9%. in FHL and 85.7 +/- 9.4%. in EBV-HLH patients, respectively. The Survival of UCBT recipients was >65%. in both FHL and EBV-HLH patients. Three Out Of four patients were alive with Successful engraftment after second UCBT. FHL patients showed a Poorer Outcome due to early treatment-related deaths (