Department of Hematology, The First Affiliated Hospital, Zhejiang University, College of Medicine, #79 Qingchun Road, 310003, Hangzhou, Zhejiang, People's Republic of China.
Institute of Hematology, Zhejiang University, Hangzhou, Zhejiang, People's Republic of China.
Int J Hematol. 2009 Oct;90(3):303-310. doi: 10.1007/s12185-009-0390-6. Epub 2009 Sep 2.
We reviewed 173 patients with an initial diagnosis of peripheral T-cell non-Hodgkin lymphoma (PTCL) and compared the patients with bone marrow involvement (BMI) to those without to have a better understanding of the clinical characteristics, treatments, survival and prognosis of PTCLs with BMI. We found that 40% (70/173) of the patients had BMI, and its frequency was 64% in angioimmunoblastic T-cell lymphoma (TCL), 46% in PTCL unspecified, 29% in anaplastic large T-cell lymphoma, 23% in extranodal NK/T-cell lymphoma and 13% in enteropathy-type TCL. In the BMI group, 36% of patients had lymphoma-associated hemophagocytic syndrome (LAHS), compared with 8% of the patients without BMI (8/103, P < 0.001). The estimated 1-year overall survival (OS) rates of patients with LAHS in the BMI and non-BMI groups were 5 and 49%, respectively. The increased levels of lactate dehydrogenase, fasting triglycerides and beta(2)-microglobulin between the BMI and non-BMI groups were not significantly different, but ferritin increased significantly and liver dysfunction-related diseases were seen more in the BMI group. As much as 51% of patients of the BMI group had anemia, compared with 27% of the patients without BMI (P = 0.001). The estimated 2-year OS rates in the two groups were 10 and 34%. The estimated 2-year OS rate of the 67 patients with BMI, who did not lose to follow-up, was 22%, compared with 38% in the non-BMI group. The median survival times of the 2 groups were 120 and 356 days. The estimated 2-year OS rate of patients treated by CHOP regimen was 9%, compared with 51% of those with intensive chemotherapy, with a significant difference (log rank P = 0.0008). The median survival time of the 14 patients subjected to chemotherapy combined with L: -asparaginase was 365 days and that of the 7 patients undergoing hemopoietic stem cell transplantation (HSCT) was 575 days. A total of 3 patients in a critical condition underwent plasmapheresis as initial therapy and achieved stable condition. We conclude that patients with PTCLs with BMI on initial diagnosis usually have hemaphagocytic syndrome and poor prognosis. BMI without lymphadenopathy is a patent clinical feature in most PTCLs. Patients with anemia on initial diagnosis in the BMI group usually have poor prognosis than those without. Intense chemotherapy, addition of L: -asparaginase in chemotherapy and HSCT are comparatively efficient treatments of PTCLs. For patients in critical conditions, plasmapheresis before chemotherapy would lower the risk and improve the tolerance to chemotherapy.
我们回顾了 173 例初始诊断为外周 T 细胞非霍奇金淋巴瘤（PTCL）的患者，并将有骨髓受累（BMI）的患者与无 BMI 的患者进行比较，以更好地了解有 BMI 的 PTCL 的临床特征、治疗、生存和预后。我们发现，40%（70/173）的患者有 BMI，其频率在血管免疫母细胞性 T 细胞淋巴瘤（TCL）中为 64%，在未特指的 PTCL 中为 46%，在间变大细胞淋巴瘤中为 29%，在结外 NK/T 细胞淋巴瘤中为 23%，在肠病型 TCL 中为 13%。在 BMI 组中，36%的患者有淋巴瘤相关噬血细胞综合征（LAHS），而无 BMI 的患者中为 8%（8/103，P<0.001）。BMI 和非 BMI 组中 LAHS 患者的估计 1 年总生存率（OS）分别为 5%和 49%。BMI 和非 BMI 组之间乳酸脱氢酶、空腹甘油三酯和β2-微球蛋白的升高水平无显著差异，但铁蛋白显著升高，BMI 组更常见肝功能相关疾病。BMI 组多达 51%的患者有贫血，而非 BMI 组为 27%（P=0.001）。两组的估计 2 年 OS 率分别为 10%和 34%。在 67 例无失访的 BMI 患者中，估计 2 年 OS 率为 22%，而非 BMI 组为 38%。两组的中位生存时间分别为 120 天和 356 天。接受 CHOP 方案治疗的患者估计 2 年 OS 率为 9%，而接受强化化疗的患者为 51%，差异有统计学意义（log rank P=0.0008）。接受化疗联合 L：-门冬酰胺酶治疗的 14 例患者的中位生存时间为 365 天，接受造血干细胞移植（HSCT）的 7 例患者的中位生存时间为 575 天。3 例病情危急的患者接受了血浆置换作为初始治疗，病情稳定。我们得出结论，初始诊断为有 BMI 的 PTCL 患者通常有噬血细胞综合征和不良预后。大多数 PTCL 中，无淋巴结病的 BMI 是一种常见的临床特征。BMI 组中初始诊断时有贫血的患者通常预后比无贫血的患者差。强化化疗、化疗中添加 L：-门冬酰胺酶和 HSCT 是治疗 PTCL 比较有效的方法。对于病情危急的患者，化疗前进行血浆置换可降低风险并提高对化疗的耐受性。
