基于依托泊苷的成人噬血细胞性淋巴组织细胞增生症治疗与高生化反应相关，但生存结果不佳。
Etoposide-based treatment of adult HLH is associated with high biochemical response but poor survival outcomes.
作者信息
Nicholson Matthew C, Naeije Leonie, Hayden Anna R, Mattman Andre, Dix David, Chen Luke Y C
机构信息
Division of Hematology, Department of Medicine University of British Columbia Vancouver BC Canada.
Division of Hematology, Oncology and Bone Marrow Transplant, Department of Paediatrics University of British Columbia Vancouver BC Canada.
出版信息
EJHaem. 2020 Jul 14;1(1):277-280. doi: 10.1002/jha2.57. eCollection 2020 Jul.
Etoposide-based treatment is the standard of care for adult HLH in many centers, yet there remains a paucity of data regarding treatment outcomes. We conducted a retrospective study of 23 adults treated with etoposide-based therapy compared to 10 pediatric HLH cases at a single center. At diagnosis, the median serum ferritin was 20,071 µg/L and 937 µg/L in adults and children, respectively; median sIL-2r was 14,524 U/mL and 4,478 U/mL. Biochemical response to treatment was high, with 21/23 adults achieving >75% reduction in serum ferritin, but one year survival was only 7/21 compared to 7/10 in pediatric cases.
在许多中心，基于依托泊苷的治疗是成人噬血细胞性淋巴组织细胞增生症（HLH）的标准治疗方法，但关于治疗结果的数据仍然很少。我们在一个中心对23例接受基于依托泊苷治疗的成人患者与10例儿童HLH病例进行了回顾性研究。诊断时，成人和儿童的血清铁蛋白中位数分别为20,071 µg/L和937 µg/L；可溶性白细胞介素-2受体（sIL-2r）中位数分别为14,524 U/mL和4,478 U/mL。治疗的生化反应率很高，23例成人中有21例血清铁蛋白降低>75%，但成人的一年生存率仅为7/21，而儿童病例为7/10。
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