title: EBV-associated post-transplantation B-cell lymphoproliferative disorder following allogenic stem cell transplantation for acute lymphoblastic leukaemia: tumor regression after reduction of immunosuppression--a case report.
abstract: Epstein-Barr virus (EBV)-associated B-cell post-transplantation lymphoproliferative disorder (PTLD) is a severe complication following stem cell transplantation. This is believed to occur as a result of iatrogenic immunosuppression leading to a relaxation of T-cell control of EBV infection and thus allowing viral reactivation and proliferation of EBV-infected B-lymphocytes. In support of this notion, reduction of immunosuppressive therapy may lead to regression of PTLD.We present a case of an 18-year-old male developing a monomorphic B-cell PTLD 2 months after receiving an allogenic stem cell transplant for acute lymphoblastic leukemia. Reduction of immunosuppressive therapy led to regression of lymphadenopathy. Nevertheless, the patient died 3 months afterwards due to extensive graft-vs.-host-disease and sepsis. As a diagnostic lymph node biopsy was performed only after reduction of immunosuppressive therapy, we are able to study the histopathological changes characterizing PTLD regression. We observed extensive apoptosis of blast cells, accompanied by an abundant infiltrate comprising predominantly CD8-positive, Granzyme B-positive T-cells. This observation supports the idea that regression of PTLD is mediated by cytotoxic T-cells and is in keeping with the observation that T-cell depletion, represents a major risk factor for the development of PTLD.
authors.affiliation: Institute of Pathology, University of Leipzig, Liebigstrasse 26, Leipzig, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Krenauer
authors.forename: Alexander
authors.initials: A
authors.lastname: Moll
authors.forename: Wolfram
authors.initials: W
authors.lastname: Pönisch
authors.forename: Nicole
authors.initials: N
authors.lastname: Schmitz
authors.forename: Gerald
authors.initials: G
authors.lastname: Niedobitek
authors.forename: Dietger
authors.initials: D
authors.lastname: Niederwieser
authors.forename: Thomas
authors.initials: T
authors.lastname: Aigner
mesh_terms: D000293:Adolescent; D001402:B-Lymphocytes / Q000276:immunology / Q000821:virology*; D001706:Biopsy; D049109:Cell Proliferation; D020031:Epstein-Barr Virus Infections / Q000209:etiology* / Q000276:immunology; D017809:Fatal Outcome; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects*; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology / Q000821:virology*; D008213:Lymphocyte Activation; D008232:Lymphoproliferative Disorders / Q000276:immunology / Q000473:pathology / Q000821:virology*; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000601:surgery*; D033581:Stem Cell Transplantation / Q000009:adverse effects*; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology / Q000821:virology; D014184:Transplantation, Homologous; D014775:Virus Activation
chemical_list: D007166:Immunosuppressive Agents