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.

Introduction

PTLD occurs as a consequence of immunosuppression in a solid-organ or hematopoietic allograft recipient. The overall incidence of PTLD following solid-organ transplant and standard T-cell repleted allogeneic bone marrow transplant is 1–3% [1] and has rarely been reported after autologous HSCT [2]. PTLD consists of a spectrum of disorders ranging from EBV-driven polyclonal proliferation resembling infectious mononucleosis to clonal EBV-positive B-cell lymphoma. In contrast to B-cell PTLD, T-cell PTLD is less frequent and is not usually associated with EBV [3]. Here, we report the case which developed clonal EBV-associated T-cell proliferation with hemophagocytosis following autologous PBSCT for relapsed AILT. This clinical presentation was compatible with previously reported fulminant T-cell LPD [4].