Kumata Hiroyuki, Nakanishi Chikashi, Murakami Keigo, Miyagi Shigehito, Fukuhara Noriko, Carreras Joaquim, Nakamura Naoya, Ichinohasama Ryo, Unno Michiaki, Kamei Takashi, Sasano Hironobu
Department of Surgery, Graduate School of Medicine, Tohoku University, 1-1 Seiryou-machi, Aobaku, Sendai, 980-8574, Japan.
Department of Pathology, Graduate School of Medicine, Tohoku University, 1-1 Seiryou-machi, Aobaku, Sendai, Miyagi, 980-8574, Japan.
Surg Case Rep. 2018 Jul 6;4(1):72. doi: 10.1186/s40792-018-0480-x.
Post-transplant lymphoproliferative disorder (PTLD) is a life-threatening complication that can be difficult to treat; moreover, determination of the pathophysiological type is difficult. We report a rare case of a patient who developed two types of Epstein-Barr virus (EBV)-negative PTLD following living donor liver transplantation (LDLT).
A 64-year-old man underwent LDLT for acute fulminant hepatitis B. Sixty-five months later, he developed EBV-negative monomorphic B cell PTLD. Reduction of immunosuppressive therapy and chemotherapy with rituximab resulted in a partial response. He received radioimmunotherapy with yttrium-90-ibritumomab tiuxetan, which was effective for all lesions, except for the splenic hilar lesion, which enlarged and seemed to penetrate the stomach. Therefore, he underwent resection of the pancreatic tail with splenectomy and partial gastrectomy. The pathological diagnosis was EBV-negative classical Hodgkin lymphoma (cHL)-type PTLD.
This patient showed an unexpected course of PTLD, from both a clinical and pathological perspective. There are no prior reports of an adult case of EBV-negative cHL-type PTLD coexisting with EBV-negative monomorphic B cell PTLD. When a strange and refractory lesion persists despite effective therapy for PTLD, we must consider the possibility of another type of PTLD within the residual lesion.
移植后淋巴组织增生性疾病(PTLD)是一种危及生命的并发症,治疗困难;此外,病理生理类型的确定也很困难。我们报告了一例罕见病例,一名患者在活体供肝移植(LDLT)后发生了两种类型的 Epstein-Barr 病毒(EBV)阴性 PTLD。
一名 64 岁男性因急性暴发性乙型肝炎接受了 LDLT。65 个月后,他发生了 EBV 阴性的单形性 B 细胞 PTLD。减少免疫抑制治疗并使用利妥昔单抗化疗后出现部分缓解。他接受了钇-90 替伊莫单抗放射免疫治疗,除脾门病变外,所有病变均有效,脾门病变增大且似乎侵犯了胃。因此,他接受了胰尾切除、脾切除和部分胃切除术。病理诊断为 EBV 阴性的经典霍奇金淋巴瘤(cHL)型 PTLD。
从临床和病理角度来看,该患者的 PTLD 病程出人意料。此前尚无成人病例报告 EBV 阴性的 cHL 型 PTLD 与 EBV 阴性的单形性 B 细胞 PTLD 共存。当对 PTLD 进行有效治疗后仍有奇怪且难治的病变持续存在时,我们必须考虑残留病变内存在另一种类型 PTLD 的可能性。