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首页> 外文期刊>Bone marrow transplantation >Bone marrow transplantation for transfused patients with severe aplastic anemia using cyclophosphamide and total lymphoid irradiation as conditioning therapy: long-term follow-up from a single center.
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Bone marrow transplantation for transfused patients with severe aplastic anemia using cyclophosphamide and total lymphoid irradiation as conditioning therapy: long-term follow-up from a single center.

机译:使用环磷酰胺和全淋巴照射作为条件疗法的重度再生障碍性贫血输血患者的骨髓移植:从单个中心进行的长期随访。

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摘要

In transfused patients with aplastic anemia, incidence of graft rejection remains significant. Seventeen transfused patients with severe aplastic anemia received BMT from HLA-identical sibling donors after conditioning with cyclophosphamide (CY, 50 mg/kg/day for 4 days) plus total lymphoid irradiation (TLI, 750 cGy in a single dose). For graft-versus-host disease (GVHD) prophylaxis one patient received methotrexate, five patients received CsA and 11 received CsA in association with methylprednisolone. All patients had sustained engraftment. The actuarial survival of patients was 76% with a median follow-up for surviving patients of 11 years (range 0.3-14.5 years). The incidence of grade II-III acute GVHD was 24%, and chronic GVHD 35%. Median Karnofsky score of surviving patients is 100 (range 90-100). Only one patient developed interstitial pneumonia. None of the patients has developed a malignancy after BMT. The role of limited field irradiation in development of malignant neoplasms after BMT for aplastic anemia is discussed. We conclude that a conditioning regimen using CY + TLI in sensitized aplastic anemia patients results in a high survival rate on long-term follow-up.
机译:在再生障碍性贫血的输血患者中,移植排斥的发生率仍然很高。 17名输血严重的再生障碍性贫血患者在接受环磷酰胺(CY,50 mg / kg /天,共4天)加总淋巴样照射(TLI,单剂量750 cGy)后接受了HLA同胞供者的BMT。对于预防移植物抗宿主病(GVHD),一名患者接受了甲氨蝶呤,五名患者接受了CsA,11名患者接受了与甲基泼尼松龙联用的CsA。所有患者均持续植入。患者的精算存活率为76%,存活患者的中位随访时间为11年(范围0.3-14.5年)。 II-III级急性GVHD的发生率为24%,而慢性GVHD的发生率为35%。存活患者的Karnofsky评分中位数为100(范围90-100)。仅一名患者发生间质性肺炎。 BMT后没有患者发生恶性肿瘤。讨论了局限性照射在再生障碍性贫血BMT后恶性肿瘤发生中的作用。我们得出的结论是,在敏感的再生障碍性贫血患者中使用CY + TLI进行调理会导致长期随访的高生存率。

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