Tahir Zabreen, Peters Julia, Leahy Kathleen, Batalini Felipe
Medicine, Division of Hematology and Oncology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.
Medicine, Division of Hematology and Oncology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA
BMJ Case Rep. 2020 Jan 2;13(1):e231831. doi: 10.1136/bcr-2019-231831.
We report the first case of double-hit (MYC and BCL-6) monomorphic post-transplant lymphoproliferative disorder in a patient status post liver transplantation. Our patient is a 71-year-old man with a past medical history of Budd-Chiari syndrome complicated by cirrhosis and hepatocellular carcinoma. He underwent a deceased donor liver transplantation 2 years prior to presentation and was maintained on tacrolimus and mycophenolate mofetil for immunosuppression. He presented with a 3-week history of classical B-symptoms. Initial workup was notable for elevated lactate dehydrogenase. Abdomen ultrasound revealed multiple hypoechoic lesions, raising suspicion for a post-transplant lymphoproliferative disorder. Biopsy showed pleomorphic large neoplastic cells throughout, consistent with a diagnosis of diffuse large B-cell lymphoma. Cytogenetics then revealed rearrangements in both MYC and BCL-6, consistent with double-hit lymphoma. His immunosuppressive regimen was subsequently tapered and he was started on DA-EPOCH-R (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab) regimen.
我们报告了首例肝移植术后发生双打击（MYC和BCL-6）单形性移植后淋巴细胞增生性疾病的病例。我们的患者是一名71岁男性，既往有布加综合征病史，并发肝硬化和肝细胞癌。他在就诊前2年接受了尸体供肝肝移植，术后使用他克莫司和霉酚酸酯进行免疫抑制治疗。他出现了3周的典型B症状病史。初步检查显示乳酸脱氢酶升高。腹部超声显示多个低回声病变，怀疑为移植后淋巴细胞增生性疾病。活检显示全是多形性大肿瘤细胞，符合弥漫性大B细胞淋巴瘤的诊断。细胞遗传学检查随后发现MYC和BCL-6均有重排，符合双打击淋巴瘤的诊断。随后逐渐减少他的免疫抑制方案，并开始使用DA-EPOCH-R（剂量调整的依托泊苷、泼尼松、长春新碱、环磷酰胺、多柔比星和利妥昔单抗）方案。
