内容紹介
A Case of Primary Testicular Diffuse Large B-Cell Lymphoma with a p53 Gene Point Mutation
Summary
A 52-year-old man with bilateral swelling in the scrotum was referred to the department of urology in our hospital in January 2013. Pathological examination of the scrotum revealed diffuse large B-cell lymphoma(DLBCL). Immunohistochemical staining revealed p53 overexpression, and polymerase chain reaction-single strand conformation polymorphism(PCR-SSCP)revealed a point mutation in exon 7 of the p53 gene. Rituximab plus cyclophosphamide, doxorubicin hydrochloride, vincristine, and prednisolone(R-CHOP)therapy and intrathecal prophylaxis were initiated. After three courses of R-CHOP therapy, high-dose cytarabine was administered, followed by peripheral blood stem cell harvesting. Busulfan, etoposide, and Ara-C(BEA)therapy was then administered, followed by autologous peripheral blood stem cell transplantation(auto-PBSCT). Primary testicular lymphoma(PTL)is a rare, clinically aggressive form of extranodal lymphoma, and there is a high incidence rate of relapse in the central nervous system(CNS). The vast majority of cases are histologically DLBCL. The p53 mutation is an independent marker of poor prognosis in patients with DLBCL treated with R-CHOP therapy. Our patient has been disease free for 17 months after auto-PBSCT with high-dose chemotherapy, which results in a greater level of penetration into the CNS.
要旨
症例は52歳,男性。2013年1月に陰嚢腫大のため当院泌尿器科へ受診した。両側除睾術後に精巣原発びまん性大細胞型B細胞リンパ腫(diffuse large B-cell lymphoma: DLBCL)と診断した。免疫染色ではp53強陽性であり,さらにp53遺伝子exon 7の点突然変異を認めた。rituximab併用CHOP(R-CHOP)療法と抗がん剤髄注を施行後に,高用量cytarabine療法による末梢血幹細胞採取とBEAを前処置とした自家末梢血幹細胞移植を行った。精巣原発リンパ腫はまれであり,進行が早く中枢神経系(central nervous system: CNS)浸潤が高率である。また大部分がDLBCLであるが,p53遺伝子変異を伴うDLBCLはR-CHOP療法に対して予後不良とされている。本症例は,CNS移行性の高い抗がん剤を組み合わせた自家末梢血幹細胞移植後17か月間,寛解を維持している。
目次
Summary
A 52-year-old man with bilateral swelling in the scrotum was referred to the department of urology in our hospital in January 2013. Pathological examination of the scrotum revealed diffuse large B-cell lymphoma(DLBCL). Immunohistochemical staining revealed p53 overexpression, and polymerase chain reaction-single strand conformation polymorphism(PCR-SSCP)revealed a point mutation in exon 7 of the p53 gene. Rituximab plus cyclophosphamide, doxorubicin hydrochloride, vincristine, and prednisolone(R-CHOP)therapy and intrathecal prophylaxis were initiated. After three courses of R-CHOP therapy, high-dose cytarabine was administered, followed by peripheral blood stem cell harvesting. Busulfan, etoposide, and Ara-C(BEA)therapy was then administered, followed by autologous peripheral blood stem cell transplantation(auto-PBSCT). Primary testicular lymphoma(PTL)is a rare, clinically aggressive form of extranodal lymphoma, and there is a high incidence rate of relapse in the central nervous system(CNS). The vast majority of cases are histologically DLBCL. The p53 mutation is an independent marker of poor prognosis in patients with DLBCL treated with R-CHOP therapy. Our patient has been disease free for 17 months after auto-PBSCT with high-dose chemotherapy, which results in a greater level of penetration into the CNS.
要旨
症例は52歳,男性。2013年1月に陰嚢腫大のため当院泌尿器科へ受診した。両側除睾術後に精巣原発びまん性大細胞型B細胞リンパ腫(diffuse large B-cell lymphoma: DLBCL)と診断した。免疫染色ではp53強陽性であり,さらにp53遺伝子exon 7の点突然変異を認めた。rituximab併用CHOP(R-CHOP)療法と抗がん剤髄注を施行後に,高用量cytarabine療法による末梢血幹細胞採取とBEAを前処置とした自家末梢血幹細胞移植を行った。精巣原発リンパ腫はまれであり,進行が早く中枢神経系(central nervous system: CNS)浸潤が高率である。また大部分がDLBCLであるが,p53遺伝子変異を伴うDLBCLはR-CHOP療法に対して予後不良とされている。本症例は,CNS移行性の高い抗がん剤を組み合わせた自家末梢血幹細胞移植後17か月間,寛解を維持している。