Division of Hematology/Medical Oncology, Department of Medicine, Taichung Veterans General Hospital, 160, Section 3, Taichung Port Road, Taichung, 407, Taiwan, Republic of China.
Ann Hematol. 2013 Nov;92(11):1529-36. doi: 10.1007/s00277-013-1784-3. Epub 2013 May 23.
Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disease. Differences between B cell and T cell lymphoma-associated HLH remain unclear, specifically clinical characteristics and survival. We retrospectively analyzed 30 lymphoma-associated HLH patients from July 2004 to October 2012. Patients were divided into B cell (n = 13) and T cell (n = 17) lymphoma groups. Patients' age, performance status, presence of Epstein-Barr virus infection, international prognostic index, presence of disseminated intravascular coagulopathy, serum triglyceride, fibrinogen, and lactate dehydrogenase levels were not significantly different between B cell and T cell lymphoma groups. HLH was an indicator for treatment resistance in patients with B cell (p = 0.048), but not T cell (p = 0.217), lymphoma. Patients in the T cell lymphoma group, however, had higher serum ferritin levels than patients in the B cell lymphoma group (11,525.6 versus 3,790.6 ng/mL; p = 0.043). The median survival time for patients in the B cell and T cell lymphoma groups was 330 and 96 days, respectively. Although the difference was not statistically significant (p = 0.273), our results suggested a trend toward a better overall survival time in patients with B cell lymphoma. This survival advantage could be at least partially due to use of rituximab (p = 0.045) for the treatment of patients with B cell lymphoma. Our results also suggested that allogeneic hematopoietic stem cell transplantation could possibly provide survival benefits to T cell lymphoma-associated HLH by graft-versus-lymphoma effect.
淋巴瘤相关噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但致命的疾病。B 细胞和 T 细胞淋巴瘤相关 HLH 之间的差异仍不清楚，特别是临床特征和生存情况。我们回顾性分析了 2004 年 7 月至 2012 年 10 月期间的 30 例淋巴瘤相关 HLH 患者。患者分为 B 细胞（n=13）和 T 细胞（n=17）淋巴瘤组。B 细胞和 T 细胞淋巴瘤组患者的年龄、表现状态、是否存在 Epstein-Barr 病毒感染、国际预后指数、是否存在弥漫性血管内凝血、血清甘油三酯、纤维蛋白原和乳酸脱氢酶水平无显著差异。HLH 是 B 细胞（p=0.048）但不是 T 细胞（p=0.217）淋巴瘤患者治疗耐药的指标。然而，T 细胞淋巴瘤组患者的血清铁蛋白水平高于 B 细胞淋巴瘤组（11525.6 与 3790.6ng/ml；p=0.043）。B 细胞和 T 细胞淋巴瘤组患者的中位生存时间分别为 330 和 96 天。尽管差异无统计学意义（p=0.273），但我们的结果表明 B 细胞淋巴瘤患者的总体生存时间有改善趋势。这种生存优势至少部分归因于利妥昔单抗（p=0.045）治疗 B 细胞淋巴瘤患者。我们的结果还表明，异基因造血干细胞移植可能通过移植物抗淋巴瘤效应为 T 细胞淋巴瘤相关 HLH 提供生存获益。
