Zinzani P L, Bendandi M, Visani G, Gherlinzoni F, Frezza G, Merla E, Manfroi S, Gozzetti A, Tura S
Institute of Hematology Seràgnoli, Policilnico S. Orsola, Blogna, Italy.
Leuk Lymphoma. 1996 Nov;23(5-6):577-82. doi: 10.3109/10428199609054867.
Lymphoblastic lymphoma (LBL) in adult patients is recognized as a particular entity in the high-grade non-Hodgkin's lymphoma (HG-NHL) group with characteristic clinical and prognostic features. Initially, polychemotherapy normally used in HG-NHL failed to produce long-term relapse-free survival because of progression disease in the CNS and in the bone marrow. Subsequently, the intensification of therapy using multimodality aggressive acute lymphoblastic leukemia (ALL) treatments led to an increase in long-term relapse-free survival. We analyzed retrospectively 53 adult patients with LBL according to the Kiel classification and the criteria by Nathwani et al. Therapeutic modifications depended upon the different times of diagnosis. Twenty-one patients received the modified L17 regimen, 13 patients were treated with the L0288 regimen, and 19 patients were submitted to the L20 protocol. There was no significant differences in CR rates among the three protocols: 48% vs 54% vs 63%, respectively. Nineteen of 29 patients who achieved CR were alive and relapse-free at a median follow-up of 84 months. Ten of the CR patients underwent autologous bone marrow transplantation (ABMT) to consolidate the first response and 7 of them are alive and relapse-free. Early stage of disease, age < 30 years, low LDH levels, the absence of leukemic phase at diagnosis, and, in particular the attainment of CR were all features of patients with good prognosis. Our study confirms the role of intensive polychemotherapeutic regimens including CNS prophylaxis, the significance of a score model of prognostic factors, and of the role of ABMT (or allogeneic bone marrow transplantation) in the treatment of adult LBL.
成人患者的淋巴细胞性淋巴瘤（LBL）被认为是高级别非霍奇金淋巴瘤（HG-NHL）组中的一种特殊实体，具有独特的临床和预后特征。最初，通常用于HG-NHL的多药化疗未能产生长期无复发生存，原因是中枢神经系统和骨髓出现疾病进展。随后，采用多模式积极急性淋巴细胞白血病（ALL）治疗方案强化治疗，使长期无复发生存率有所提高。我们根据 Kiel 分类法和 Nathwani 等人的标准，对 53 例成年 LBL 患者进行了回顾性分析。治疗方案的调整取决于诊断时间的不同。21 例患者接受改良的 L17 方案，13 例患者接受 L0288 方案治疗，19 例患者采用 L20 方案。三种方案的完全缓解（CR）率无显著差异：分别为 48%、54%和 63%。29 例达到 CR 的患者中有 19 例在中位随访 84 个月时存活且无复发。10 例 CR 患者接受了自体骨髓移植（ABMT）以巩固首次缓解，其中 7 例存活且无复发。疾病早期、年龄<30 岁、乳酸脱氢酶（LDH）水平低、诊断时无白血病期，尤其是达到 CR 是预后良好患者的所有特征。我们的研究证实了包括中枢神经系统预防的强化多药化疗方案的作用、预后因素评分模型的意义以及 ABMT（或异基因骨髓移植）在成人 LBL 治疗中的作用。
