Cooper Maureen A, Akard Luke P, Thompson James M, Dugan Michael J, Jansen Jan
Indiana Oncology-Hematology Consultants, Indianapolis, Indiana 46107, USA.
Am J Hematol. 2005 Jan;78(1):33-6. doi: 10.1002/ajh.20214.
A 38-year-old male with progressive myeloproliferative variant of hypereosinophilic syndrome (HES) underwent allogeneic bone marrow transplantation from a matched unrelated donor. The preparative regimen consisted of TBI, cytarabine, and cyclophosphamide. The graft was T-cell-depleted. The patient had slow, but complete, hematologic recovery, and all cells were shown by VNTR analysis to be of donor origin. Five months after transplant, the patient developed prominent eosinophilia (peak 4.1 x 10(9)/L) with dermatographism and very high IL-5 levels. Eosinophils isolated to purity by cell sorting were all of donor origin. Mild increase in immunosuppression led to a normalization of eosinophil count after about 6 months. The patient is now 6 years after transplant, off all medications, and without evidence of disease. Allogeneic stem-cell transplantation is a potentially curative therapy for HES.
一名患有嗜酸性粒细胞增多综合征(HES)进行性骨髓增殖变异型的38岁男性接受了来自匹配无关供者的异基因骨髓移植。预处理方案包括全身照射、阿糖胞苷和环磷酰胺。移植物进行了T细胞去除。患者血液学恢复缓慢但完全,通过可变数目串联重复序列(VNTR)分析显示所有细胞均来自供者。移植后5个月,患者出现显著的嗜酸性粒细胞增多(峰值为4.1×10⁹/L),伴有皮肤划痕症和非常高的白细胞介素-5水平。通过细胞分选分离至纯度的嗜酸性粒细胞均来自供者。免疫抑制的轻度增加导致约6个月后嗜酸性粒细胞计数恢复正常。该患者目前移植后已6年,停用所有药物,且无疾病证据。异基因干细胞移植是HES的一种潜在治愈性疗法。