利妥昔单抗联合化疗治疗原发CD5阳性弥漫大B细胞淋巴瘤二例并文献复习

目的 探讨原发CD5阳性(CD5+)弥漫大B细胞淋巴瘤的生物学特性及利妥昔单抗联合不同化疗方案的治疗效果.方法 总结既往报道的1例及山西医科大学第二医院血液科收治的1例CD5+弥漫大B细胞淋巴瘤患者的临床特点,分析骨髓及淋巴结细胞形态学、流式细胞术、免疫组织化学和分子生物学特征,评估利妥昔单抗联合不同化疗方案的治疗反应,并复习相关文献.结果 例1患者初诊明确诊断为原发CD5+弥漫大B细胞淋巴瘤(白血病期),伴复杂核型,利妥昔单抗联合VCTP(4周方案)诱导化疗1个疗程后骨髓达完全缓解,脾脏及淋巴结明显缩小,后予R-VCTP(2周方案)×1、R-Hyper-CVAD×3、R-HD-MTX×4方案巩固强化治疗8次,阿糖胞苷联合甲氨蝶呤鞘内注射10次,随访21个月,疾病持续缓解,无中枢神经系统侵犯表现.例2为高龄患者,明确诊断为原发CD5+弥漫大B细胞淋巴瘤(鼻型),给予利妥昔单抗联合VP方案及单药治疗3个疗程,鼻骨肿物完全回缩,局部骨质修复;自行停药半年后出现多发皮肤病变,给予利妥昔单抗联合COP方案再次化疗后皮肤病变缓解,但磁共振成像提示脑实质浸润,放疗后症状缓解,浸润灶大部分吸收;随访21个月,疾病稳定.结论 原发CD5+弥漫大B细胞淋巴瘤易合并结外病变,以骨髓、皮肤及中枢神经系统受累多见.利妥昔单抗联合急性淋巴细胞白血病整体治疗及鞘内注射方案可能有助于降低转移性中枢神经系统病变发生率,提高无病生存率.

Rituximab combined with chemotherapy for treatment of primary CD5 positive diffuse large B-cell lymphoma: report of two cases and review of literature

Objective To explore the biological characteristics of primary CD5 positive diffuse large B-cell lymphoma (DLBCL) and treatment effects of rituximab combined with different chemotherapy regimens. Methods The clinical features of 2 CD5 positive DLBCL patients (1 case with early report and 1 case with newly treated) in department of hematology of the Second Hospital of Shanxi Medical University were summarized, including bone marrow, lymph nodes morphology, flow cytometry, immunohistochemistry, and molecular biology characteristics. The treatment response of rituximab combined with different chemotherapy regimens was evaluated, and the literatures were reviewed. Results First patient was diagnosed as primary CD5 positive DLBCL with complex karyotype (leukemia stage). After the first induction chemotherapy with rituximab and VCTP regimens (4 weeks), the patient achieved complete remission of bone marrow. Spleen and lymph nodes also were reduced significantly. Eight consolidation therapy including R-VCTP regimen (2 weeks), R-Hyper-CVAD regimens for 3 courses and R-HD-MTX regimens for 4 courses were sequentially given. Cytarabine combined with and methotrexate (MTX) was performed 10 times of intrathecal injection. After the follow-up of 21 months, the disease sustained remission without central nervous system (CNS) invasiveness. Second elderly patient was diagnosed as primary CD5 positive DLBCL (nasal type), who received induction chemotherapy regimen of rituximab combined with vincristine and prednisone for 3 courses. Nasal tumor symptoms completely relieved after subsequent treatments and the local sclerotin restored. However, multiple skin lesions appeared after half a year, which were quickly alleviated after treatment of rituximab combined with COP regimen. Magnetic resonance imaging (MRI) showed that the brain parenchymal infiltration appeared. Related symptoms gradually relieved after radiotherapy and imageology also showed that infiltrates were mostly absorbed. The patient was followed up for 21 months and had the stable disease. Conclusions Primary CD5 positive DLBCL is a unique subset presented with more extranodal lesions, which occur commonly in bone marrow, skin and CNS. Rituximab combined with acute lymphoblastic leukemia chemotherapy and intrathecal chemotherapy may reduce incidence of metastatic central lesions , and improve the disease free survival rate.