Fujishima Takashi, Kawabata Yoshinari, Michishita Yoshihiro, Kitabayashi Atushi, Takahashi Naoto
Department of Hematology, Akita Kosei Medical Center.
Department of Hematology, Nephrology, and Rheumatology, Akita University.
Rinsho Ketsueki. 2022;63(10):1386-1391. doi: 10.11406/rinketsu.63.1386.
Plasmablastic lymphoma (PBL) is a rare variant of diffuse large B-cell lymphoma that is frequently associated with HIV infection or other immunodeficiencies. We present a case of HIV-negative primary testicular PBL with long-term complete remission (CR) and successful treatment with bortezomib in combination with EPOCH (V-EPOCH). Because of rapidly increasing right testicular swelling, an 86-year-old man without immunodeficiencies was admitted to our hospital. Following that, a right high orchiectomy was performed. Histopathological and immunohistochemical analyses revealed diffuse infiltration of plasmablastic lymphocytes, which were positive for CD38, CD138, CD56, MUM1, lambda, EBER, and MYC respectively, but negative for CD20. The MIB-1 index was 90%. FDG abnormal uptake was discovered on PET/CT at systemic lymph nodes. There was no abnormal cell infiltration in either the bone marrow or cerebral spinal fluid. He was diagnosed with PBL, clinical-stage IIIE-A, IPI high-intermediate risk. He achieved durable CR more than 30 months after the diagnosis after six courses of V-EPOCH, followed by intrathecal prophylaxis (IT) and adjuvant radiation therapy (total 30 Gy). Although PBL shows an aggressive clinical course and poor prognosis, adequate therapeutic approaches for PBL have not been established due to the rarity of this disease. According to our findings, V-EPOCH combined with IT and adjuvant radiotherapy appeared to be feasible and effective as a frontline treatment for elderly patients with primary testicular PBL.
浆母细胞性淋巴瘤（PBL）是弥漫性大B细胞淋巴瘤的一种罕见变体，常与HIV感染或其他免疫缺陷相关。我们报告一例HIV阴性的原发性睾丸PBL病例，该患者长期完全缓解（CR），并通过硼替佐米联合EPOCH（V-EPOCH）成功治疗。因右侧睾丸迅速肿大，一名无免疫缺陷的86岁男性入住我院。随后，进行了右侧高位睾丸切除术。组织病理学和免疫组织化学分析显示浆母细胞性淋巴细胞弥漫浸润，这些细胞分别对CD38、CD138、CD56、MUM1、λ、EBER和MYC呈阳性，但对CD20呈阴性。MIB-1指数为90%。PET/CT显示全身淋巴结有FDG异常摄取。骨髓和脑脊液均无异常细胞浸润。他被诊断为PBL，临床分期为IIIE-A，国际预后指数（IPI）为高中危。在接受六个疗程的V-EPOCH治疗，随后进行鞘内预防（IT）和辅助放疗（共30 Gy）后，他在诊断后30多个月实现了持久的CR。尽管PBL临床病程侵袭性强且预后差，但由于该疾病罕见，尚未确立足够的治疗方法。根据我们的研究结果，V-EPOCH联合IT和辅助放疗作为老年原发性睾丸PBL患者的一线治疗似乎可行且有效。
