title: Fulminant Epstein-Barr virus (EBV)-associated T-cell lymphoproliferative disorder with hemophagocytosis following autologous peripheral blood stem cell transplantation for relapsed angioimmunoblastic T-cell lymphoma.
abstract: Post-transplant lymphoproliferative disorder (PTLD) is a complication that can develop after either solid-organ or hematopoietic stem cell transplantation (HSCT). T-cell PTLD is a rare disorder, especially following autologous HSCT. Here we report a case of T-cell PTLD which occurred after autologous peripheral blood stem cell transplantation (PBSCT) for relapsed angioimmunoblastic T-cell lymphoma (AILT). Three months after the transplant, the patient developed fever with elevated plasma Epstein-Barr virus (EBV)-PCR values. The patient subsequently developed pneumonitis, hepatomegaly and marked pancytopenia due to hemophagocytosis. The patient died of multi-organ failure, despite antiviral and steroid pulse therapy. Our post-mortem study confirmed the marked proliferation of EBV-infected T-cells that differed from the original AILT clone and macrophages/histiocytes were observed in the marrow, liver, lymph nodes and lungs. Phagocytosis was most evident in the bone marrow. The patient's AILT remained in complete remission. To the best of our knowledge, this is the first case of fulminant EBV-associated T-cell lymphoproliferative disorder (LPD) following autologous HSCT.
authors.affiliation: Department of Medicine, Division of Hematology, Keio University School of Medicine, Japan. nawaya@sc.itc.keio.ac.jp
authors.forename: Norihiro
authors.initials: N
authors.lastname: Awaya
authors.forename: Akiko
authors.initials: A
authors.lastname: Adachi
authors.forename: Taisuke
authors.initials: T
authors.lastname: Mori
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kamata
authors.forename: Jin
authors.initials: J
authors.lastname: Nakahara
authors.forename: Kenji
authors.initials: K
authors.lastname: Yokoyama
authors.forename: Taketo
authors.initials: T
authors.lastname: Yamada
authors.forename: Masahiro
authors.initials: M
authors.lastname: Kizaki
authors.forename: Michiie
authors.initials: M
authors.lastname: Sakamoto
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Ikeda
authors.forename: Shin-ichiro
authors.initials: S
authors.lastname: Okamoto
mesh_terms: D015703:Antigens, CD / Q000096:biosynthesis; D015214:Antigens, Differentiation, Myelomonocytic / Q000096:biosynthesis; D017252:CD3 Complex / Q000096:biosynthesis; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017809:Fatal Outcome; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000150:complications / Q000473:pathology / Q000628:therapy*; D016411:Lymphoma, T-Cell, Peripheral / Q000150:complications / Q000473:pathology / Q000628:therapy*; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000473:pathology / Q000821:virology; D008297:Male; D008875:Middle Aged; D036102:Peripheral Blood Stem Cell Transplantation / Q000009:adverse effects*; D012008:Recurrence; D012074:Remission Induction; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology / Q000821:virology; D014182:Transplantation, Autologous; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D017252:CD3 Complex; C067980:CD68 antigen, human; D000097382:CD68 Molecule