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摘要: 目的 分析浆母细胞淋巴瘤(PBL)患者的临床和病理特征、鉴别诊断、治疗及预后。方法 回顾性分析青岛大学附属医院2013年9月至2020年4月确诊并治疗的7例PBL患者的临床资料，并进行文献复习。结果 7例PBL患者均为HIV阴性，其中男3例，女4例，中位年龄63(51~75)岁。1例为腹股沟淋巴结起病，其余6例为结外器官受累起病，初诊Ann Arbor分期Ⅳ期5例，3例存在B症状。所有患者瘤细胞弥漫表达CD38，其次为CD138和MUM-1，B细胞标志物CD20少见，EBER阳性1例。3例患者接受原发病灶切除术，5例患者以CHOP样方案作为一线化疗方案，其中1例完全缓解并接受自体造血干细胞移植，6例死亡。7例患者中位生存时间7.2(0.8~46.7)个月。结论 PBL侵袭性强，易累及结外组织，多数患者确诊时处于晚期；尚无统一治疗方案，对化疗反应差，死亡率高；有研究报道硼替佐米的应用和自体造血干细胞移植在一定程度上有助于延长患者生存。Abstract: Objective To analyze the clinicopathologic characteristics, differential diagnosis, treatment and prognosis of patients with plasmablastic lymphoma(PBL).Methods We collected and organized the clinical data of 7 patients with PBL diagnosed in the Affiliated Hospital of Qingdao University from September 2013 to April 2020. The clinicopathologic characteristics, immunophenotypes, treatment and prognosis were retrospectively analyzed. Related literatures were reviewed.Results Seven PBL patients including 3 males and 4 females were all HIV negative, with a median age of 63 years(range 51-75). Inguinal lymph node involvement as origin was in 1 case and the remaining 6 cases had onset of extranodal organ involvement. According to Ann Arbor staging, 5 cases were at stage Ⅳ and 3 cases had B symptoms. CD38 as plasma cell marker was diffusely expressed in tumor cells of all patients, followed by CD138 and MUM-1. CD20 as B cell marker was rare. EBER was positive in only 1 case. Three patients underwent primary lesion resection, 5 patients received CHOP-like regimen as the first-line chemotherapy regimen. One case had complete remission after chemotherapy and received autologous hematopoietic stem cell transplantation, the others all died. The median survival time of 7 patients was 7.2 months(range 0.8-46.7).Conclusion PBL is a rare and aggressive lymphoma that easily affects extranodal tissues, not sensitive to chemotherapy and has high mortality. Most patients are diagnosed at advanced disease stage. Although no uniform treatment plan exists, application of bortezomib and autologous hematopoietic stem cell transplantation can help prolong the survival of patients to a certain extent.
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Key words:
- plasmablastic lymphoma /
- clinical features /
- differential diagnosis /
- treatment /
- prognosis
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表 1 7例HIV阴性PBL患者的临床特点、治疗方案和转归
病例 年龄
/岁性别 Ann Arbor分期 IPI评分 起病部位 受累部位 伴随疾病 治疗方案△ 疗效 PFS/
月OS/
月转归 1 69 男 Ⅳ期B 4 肝脏 肝脏、淋巴结 结肠癌 经皮肝动脉化疗栓塞术 无法评估 无法评估 1.0 死亡 2 75 女 Ⅳ期B 4 腹股沟淋巴结 淋巴结、骨髓 糖尿病、慢性肾盂肾炎、冠心病、脑栓塞 腹股沟肿物切除 无法评估 无法评估 7.9 死亡 3 61 女 Ⅳ期A 3 下颌骨 下颌骨、骨髓 无 CHOP×2
CHOPE×2PR后进展 5.9 7.2 死亡 4 57 男 Ⅱ期A 1 鼻咽 鼻咽、淋巴结 无 鼻咽颅底肿物切除术
CHOP×3
CHOPE×3
VADPT×3
ASCTCR 46.7 46.7 存活 5 61 女 Ⅳ期A 5 胃 胃、脾脏、肝脏、淋巴结 糖尿病、脾切除术 根治性全胃切除术
CHOP×6
CHOPE×3
GemOX×1PR后进展 7.2 8.0 死亡 6 51 女 Ⅳ期A 3 颊部 颌下腺、骨骼、淋巴结 重症肌无力、甲亢 C2PET×1 PD 无法评估 0.8 死亡 7 67 男 Ⅱ期B 3 膈肌 膈肌、淋巴结 左肺切除术、干燥综合征 CODE×2 PR后进展 0.7 1.2 死亡 △治疗方案中，CHOP：环磷酰胺+多柔比星+长春新碱+泼尼松；CHOPE：CHOP+依托泊苷；VADPT：长春地辛+吡柔比星+地塞米松+顺铂+沙利度胺；ASCT：自体造血干细胞移植；GemOX：吉西他滨+奥沙利铂；C2PET：环磷酰胺+西达本胺+泼尼松+依托泊苷+沙利度胺；CODE：环磷酰胺+长春新碱+地塞米松+依托泊苷。 
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表 2 7例HIV阴性PBL患者的免疫表型
病例 CD79α CD38 CD138 CD20 CD3 CD56 MUM1 Ki-67 Bcl-2 Bcl-6 EBER 1 无资料 + + - - NA NA 90% 无资料 无资料 - 2 + + - + - - - 60% + + - 3 - + + - - - + 90% 无资料 - - 4 无资料 + - - - - + 50% + + + 5 无资料 + + - - +/- + 80% - - - 6 - + - - - NA - 60% 无资料 无资料 - 7 无资料 - + - - - + 90% 无资料 无资料 -
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| 引用本文: |
姜骁娜, 史雪, 侯峰, 等. 7例浆母细胞淋巴瘤临床特征分析[J]. 临床血液学杂志, 2022, 35(1): 63-67. doi: 10.13201/j.issn.1004-2806.2022.01.012
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| Citation: |
JIANG Xiaona, SHI Xue, HOU Feng, et al. Clinical features analysis of 7 cases with plasmablastic lymphoma[J]. J Clin Hematol, 2022, 35(1): 63-67. doi: 10.13201/j.issn.1004-2806.2022.01.012
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