Gaziev D, Giardini C, Galimberti M, Lucarelli G, Angelucci E, Polchi P, Baronciani D, Erer B, Sotti G
Divisione di Ematologia e Centro Trapianti di Midollo Osseo di Muraglia, Azienda Ospedaliera di Pesaro, Italy.
Bone Marrow Transplant. 1999 Aug;24(3):253-7. doi: 10.1038/sj.bmt.1701887.
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.
在接受输血的再生障碍性贫血患者中，移植物排斥的发生率仍然很高。17例接受输血的重型再生障碍性贫血患者在接受环磷酰胺（CY，50mg/kg/天，共4天）加全身淋巴照射（TLI，单次剂量750cGy）预处理后，接受了来自HLA相同同胞供者的骨髓移植。为预防移植物抗宿主病（GVHD），1例患者接受了甲氨蝶呤，5例患者接受了环孢素，11例患者接受了环孢素联合甲基泼尼松龙。所有患者均实现持续植入。患者的精算生存率为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%，慢性GVHD为35%。存活患者的中位卡诺夫斯基评分是100分（范围90 - 100）。仅一名患者发生间质性肺炎。骨髓移植后没有患者发生恶性肿瘤。讨论了有限野照射在再生障碍性贫血骨髓移植后恶性肿瘤发生中的作用。我们得出结论，在致敏的再生障碍性贫血患者中使用CY + TLI的预处理方案在长期随访中可获得高生存率。
