Blood and Marrow Transplant & Cellular Therapy Program, Medical College of Wisconsin, Milwaukee, Wisconsin; Division of Hematology/Oncology, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin.
Division of Hematology/Oncology, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin.
Biol Blood Marrow Transplant. 2020 May;26(5):893-901. doi: 10.1016/j.bbmt.2020.01.010. Epub 2020 Jan 23.
Allogeneic hematopoietic cell transplantation (allo-HCT) is the only curative treatment modality for primary myelofibrosis (MF) and related myeloproliferative neoplasms. Older age at diagnosis and age-related comorbidities make most patients ineligible for allo-HCT, given concerns for nonrelapse mortality (NRM). Here we report the outcomes of 37 consecutive recipients of allo-HCT for MF performed at a single center between 2009 and 2018 with a standardized institutional protocol. Most patients received ruxolitinib before HCT (n = 32), and those with splenomegaly >22 cm received pretransplantation splenic irradiation. The median age at HCT was 60 years (range, 40 to 74 years), and 68% of the cohort carried a JAK2 driver mutation. All patients received fludarabine/busulfan-based conditioning; 22 patients (59%) received a reduced-intensity conditioning regimen. All patients received peripheral blood grafts, from a matched sibling donor in 16 patients (43%), an unrelated donor in 20 patients, and a haploidentical-related donor in 1 patient. Sixty-one percent had a Hematopoietic Cell Transplantation Comorbidity Index ≥3, 40% had a Karnofsky Performance Status score <90, and 24% had a high-risk DIPSS Plus score. With a median follow-up of 40.2 months (range, 16.9 to 115 months), the 3-year overall survival and relapse-free survival were 81.1% (95% confidence interval [CI], 64.4% to 90.5%) and 78.4% (95% CI, 61.4% to 88.5%), respectively. Only 2 patients relapsed/progressed after transplant. NRM at 2 years was 16.2% (95% CI, 6.5% to 29.9%). All patients engrafted. Sixteen patients were treated with ruxolitinib post-transplantation for graft-versus-host disease, graft rejection/relapse, or persistent MF. These results suggest that pretransplantation ruxolitinib, fludarabine/busulfan-based conditioning, and splenic management are keys to improved transplantation outcomes in patients undergoing allo-HCT for MF.
异基因造血细胞移植(allo-HCT)是原发性骨髓纤维化(MF)和相关骨髓增生性肿瘤的唯一根治性治疗方法。由于担心非复发死亡率(NRM),大多数年龄较大且存在与年龄相关的合并症的患者不符合 allo-HCT 的条件。在这里,我们报告了 2009 年至 2018 年期间在一家中心进行的 37 例连续接受 MF allo-HCT 的患者的结果,这些患者使用了标准化的机构方案。大多数患者在 HCT 前接受了鲁索替尼治疗(n=32),且脾脏肿大>22cm 的患者接受了移植前脾照射。HCT 时的中位年龄为 60 岁(范围为 40 至 74 岁),68%的患者携带 JAK2 驱动突变。所有患者均接受氟达拉滨/白消安为基础的预处理方案;22 例患者(59%)接受了低强度预处理方案。所有患者均接受外周血移植物,16 例患者(43%)接受了同胞供体,20 例患者接受了无关供体,1 例患者接受了单倍体相关供体。61%的患者有造血细胞移植合并症指数(HCT-CI)≥3,40%的患者有卡氏功能状态评分(KPS)<90,24%的患者有高危 DIPSS Plus 评分。中位随访时间为 40.2 个月(范围为 16.9 至 115 个月),3 年总生存率和无复发生存率分别为 81.1%(95%置信区间 [CI],64.4%至 90.5%)和 78.4%(95% CI,61.4%至 88.5%)。仅有 2 例患者在移植后复发/进展。2 年 NRM 为 16.2%(95%CI,6.5%至 29.9%)。所有患者均植入。16 例患者在移植后因移植物抗宿主病、移植物排斥/复发或持续性 MF 接受了鲁索替尼治疗。这些结果表明,移植前鲁索替尼、氟达拉滨/白消安为基础的预处理方案和脾脏管理是改善接受 allo-HCT 治疗的 MF 患者移植结果的关键。