Liu Yao-Chung, Gau Jyh-Pyng, Yu Yuan-Bin, Hong Ying-Chung, Yen Chueh-Chuan, Liu Chun-Yu, Chao Ta-Chung, Hsiao Liang-Tsai, Liu Jin-Hwang, Chiou Tzeon-Jye, Tzeng Cheng-Hwai
Division of Hematology and Oncology, Department of Medicine, Taipei Veterans General Hospital, Taiwan.
Intern Med. 2014;53(2):95-101. doi: 10.2169/internalmedicine.53.0967. Epub 2012 Mar 1.
Primary bone lymphoma is a rare disorder, accounting for less than 1% of all cases of malignant lymphoma. Primary bony diffuse large B-cell lymphoma (PBDLBCL) is the most common histological type. In our study, a favorable response and lower risk of emergent surgery were observed following the administration of systemic chemotherapy with or without rituximab.
Patients diagnosed with malignant lymphoma at our hospital between 2000 and 2011 were evaluated for PBDLBCL. Pertinent data, including the clinical presentation, histological type, treatment modalities, long-term outcome, survival curve and prognostic factors, were analyzed.
Twenty-four patients with a median age of 63 years were diagnosed with PBDLBCL. A complete response (CR) was achieved in 58.4% (n=14) of the patients. With treatment of the disease, nine of 10 patients with initially impending pathological bone fractures ultimately did not undergo surgery. The median follow-up duration was 48 months. Two patients experienced disease relapse. In a Kaplan-Meier analysis, the 5-year overall survival (OS) and disease-free survival (DFS) rates were 66.7% and 77.8%, respectively. In the univariate analyses, the significantly favorable prognostic factors for OS were an International Prognostic Index (IPI) score of <3, an age of ≤60 years, a performance status with an Eastern Cooperative Oncology (ECOG) score of <2, a CR and chemotherapy ≥6 cycles. For DFS, a CR was the only favorable factor. In the multivariate analysis, a CR was the only independent factor for both OS and DFS.
Our study confirms the good prognosis of this rare disorder. Once a CR is achieved, even elderly patients may exhibit long-term survival, possibly obviating the need for surgery for less severe bone lesions.
原发性骨淋巴瘤是一种罕见疾病，占所有恶性淋巴瘤病例的比例不到1%。原发性骨弥漫性大B细胞淋巴瘤（PBDLBCL）是最常见的组织学类型。在我们的研究中，观察到在使用或不使用利妥昔单抗进行全身化疗后，患者有良好的反应且急诊手术风险较低。
对2000年至2011年期间在我院诊断为恶性淋巴瘤的患者进行PBDLBCL评估。分析相关数据，包括临床表现、组织学类型、治疗方式、长期结局、生存曲线和预后因素。
24例中位年龄为63岁的患者被诊断为PBDLBCL。58.4%（n = 14）的患者实现了完全缓解（CR）。随着疾病的治疗，10例最初有即将发生病理性骨折的患者中有9例最终未接受手术。中位随访时间为48个月。2例患者出现疾病复发。在Kaplan-Meier分析中，5年总生存率（OS）和无病生存率（DFS）分别为66.7%和77.8%。在单因素分析中，OS的显著有利预后因素为国际预后指数（IPI）评分<3、年龄≤60岁、东部肿瘤协作组（ECOG）评分<2的体能状态、CR以及化疗≥6周期。对于DFS，CR是唯一的有利因素。在多因素分析中，CR是OS和DFS的唯一独立因素。
我们的研究证实了这种罕见疾病的良好预后。一旦实现CR，即使是老年患者也可能长期生存，可能无需对不太严重的骨病变进行手术。
