Onitilo A A, Lazarchick J, Brunson C Y, Frei-Lahr D, Stuart R K
Department of Medicine, Hematology/Oncology Division, Medical University of South Carolina, 96 Jonathon Lucas Street, Charleston, SC 29425, USA.
Transplant Proc. 2003 Dec;35(8):3089-92. doi: 10.1016/j.transproceed.2003.10.085.
As the life expectancy of patients with homozygous sickle cell anemia (SCA) improves, SCA care providers are confronted with diseases of the adult SCA population rarely seen before. We report here a 40-year-old woman with SCA who developed diffuse large B-cell non-Hodgkin's lymphoma (NHL) that was treated with eight cycles of chemotherapy consisting of cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide (CHOPE), without complete remission. She subsequently underwent high-dose cyclophosphamide and total-body irradiation followed by autologous bone marrow transplantation (BMT). To reduce the risk of sickle cell crisis precipitated by G-CSF, she underwent hypertransfusion to maintain a low % hemoglobin S throughout her treatment course. Although she has required iron chelation therapy and shows no sign of modification of her underlying SCA, she remains in remission from NHL 12 years posttransplant. To our knowledge, this is the first reported case of autologous BMT in a patient with SCA. Our patient illustrates that SCA in itself does not preclude autologous stem cell transplantation for lymphoma in selected patients, and this report should encourage others to consider autologous BMT in adults with SCA where it represents a lifesaving therapy for malignant diseases.
随着纯合子镰状细胞贫血(SCA)患者预期寿命的延长,SCA护理人员面临着成年SCA患者群体中以前很少见到的疾病。我们在此报告一名40岁的SCA女性患者,她患上了弥漫性大B细胞非霍奇金淋巴瘤(NHL),接受了由环磷酰胺、阿霉素、长春新碱、泼尼松和依托泊苷(CHOPE)组成的八个周期化疗,但未完全缓解。随后她接受了大剂量环磷酰胺和全身照射,然后进行了自体骨髓移植(BMT)。为降低G-CSF引发镰状细胞危象的风险,她在整个治疗过程中进行了换血治疗以维持低水平的血红蛋白S。尽管她需要进行铁螯合治疗,且其潜在的SCA没有改善迹象,但移植后12年她的NHL仍处于缓解状态。据我们所知,这是首例关于SCA患者自体BMT的报道病例。我们的患者表明,SCA本身并不排除在选定患者中对淋巴瘤进行自体干细胞移植,本报告应鼓励其他人在成年SCA患者中考虑自体BMT,因为这对恶性疾病来说是一种挽救生命的治疗方法。