Classical Hodgkin Lymphoma-type Post-Transplant Lymphoproliferative Disorder in a Pediatric Liver Transplant Patient

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Sujin Choi¹, Jung Yoon Choi¹^,2, Kyung Taek Hong¹^,2, Che Ry Hong¹^,2, Hyoung Jin Kang¹^,2, Kyung Duk Park¹^,2, Jae Sung Ko¹, Kyung Suk Suh³ , Hee Young Shin¹^,2

Seoul National Univesity College of Medicine Department of Pediatrics¹

Seoul National Univesity College of Medicine Cancer Research Institute ²

Seoul National Univesity College of Medicine Department of Surgery³

최수진¹, 최정윤¹^,2, 홍경택¹^,2, 홍채리¹^,2, 강형진¹^,2, 박경덕¹^,2, 고재성¹, 서경석³ , 신희영¹^,2

서울대학교 의과대학 소아청소년과¹

서울대학교 의과대학 암연구소²

서울대학교 의과대학 외과³

Abstract

Introduction: Post-transplant lymphoproliferative disorder ( PTLD) is a heterogeneous group of diseases with abnormal proliferation of lymphoid tissue and is well recognized as a severe complication of immunosuppression in transplant patients. PTLD is generally associated with the use of immunosuppressive treatments, T-cell depleting agents, Epstein–Barr virus (EBV) infection and subsequent B-cell hyperplasia. Classical Hodgkin lymphoma (CHL) type PTLD is a rare subtype comprising less than 10% of all PTLD. Case: We describe a rare case of a 17 year old girl diagnosed with CHL type PTLD stage IV, 6 years after liver transplantation (LT) for post-Kasai biliary atresia. The patient presented with left pelvic, low back pain and fever. Thorough evaluation revealed multiple lymph node involvements on both sides of the diaphragm with suspicious involvement of L1 vertebrae. Inguinal lymph node biopsy was done and neoplastic cells were Epstein-Barr encoded RNA in situ hybridization positive and expressed CD3, CD20, CD30, CD15 and Ki-67. The pathologic type was nodal sclerosis. The patient was on immunosuppressive treatment consisting of prednisolone and tacrolimus. At the time of diagnosis, she was EBV sero-positive with a EB viral load of 107,412 copies/mL in whole blood. The patient was treated with standard CHL (CCG 5942 group 3) combination chemotherapy (high dose cytarabine, etoposide, cyclophosphamide, vincristine, procarbazine, prednisolone, doxorubicin, bleomycin and vinblastine) and Rituximab with dose reduction of immunosuppressant. Although the last cycle of chemotherapy was not performed due to prolonged grade 3 thrombocytopenia, the patient still remains in complete remission 8 months after her last therapy. Conclusion: To our best knowledge, this is the first pediatric case report of a successfully treated HL type PTLD after LT in the post millennium era. Multicenter studies are required to further help characterize the treatment strategies and related toxicities, prognostic factors and screening modalities of this uncommon entity.

Keywords: Classical Hodgkin lymphoma, Post-transplant lymphoproliferative disorder , Liver Transplantation