Singhal S, Powles R, Treleaven J, Pollard C, Lumley H, Mehta J
Royal Marsden Hospital Sutton, Surrey, UK.
Bone Marrow Transplant. 1995 Dec;16(6):743-6.
The published experience of allogeneic bone marrow transplantation for primary myelofibrosis (PMF) is limited. Three patients (24-49 years) with PMF received allogeneic marrow grafts from HLA-identical sibling donors after conditioning with 110 mg/m2 melphalan and 1050 cGy total-body irradiation (TBI). Donor marrow was not depleted of T cells, and graft-versus-host disease (GVHD) prophylaxis comprised cyclosporine and methotrexate. None of the patients was splenectomized prior to the transplant. Two patients received G-CSF post-transplant to hasten neutrophil recovery. One patient died of multi-organ failure 23 days post-transplant. Hematopoietic recovery was relatively slow in the other two who had gradual resolution of the marrow fibrosis over several months. One of the two died of overwhelming pneumococcal sepsis within 2 weeks of stopping prophylactic penicillin 31 months post-transplant. The other patient is alive and well 20 months post-transplant with a Karnofsky score of 100% and no fibrosis of the marrow. We conclude that PMF is correctable by allogeneic BMT. Hematologic recovery post-transplant is slow, but counts may normalize with time without the need for splenectomy.
异基因骨髓移植治疗原发性骨髓纤维化（PMF）的已发表经验有限。3例（24 - 49岁）PMF患者在接受110 mg/m²美法仑和1050 cGy全身照射（TBI）预处理后，接受了来自HLA相同同胞供体的异基因骨髓移植。供体骨髓未进行T细胞去除，移植物抗宿主病（GVHD）预防措施包括环孢素和甲氨蝶呤。移植前所有患者均未行脾切除术。2例患者移植后接受粒细胞集落刺激因子（G-CSF）以加速中性粒细胞恢复。1例患者在移植后23天死于多器官功能衰竭。另外2例患者造血恢复相对缓慢，骨髓纤维化在数月内逐渐缓解。其中1例在移植后31个月停用预防性青霉素后2周内死于暴发性肺炎球菌败血症。另1例患者在移植后20个月时存活且状况良好，卡氏评分100%，骨髓无纤维化。我们得出结论，异基因骨髓移植可纠正PMF。移植后血液学恢复缓慢，但随着时间推移血细胞计数可能恢复正常，无需行脾切除术。
