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Hematopoietic stem cell transplantation in patients with lymphomatoid granulomatosis: a European group for blood and marrow transplantation report.

Abstract
Lymphomatoid granulomatosis (LG) is a very rare, Epstein-Barr virus-associated lymphoproliferative disorder of B cells. Prognosis is poor, particularly after relapse and no curative treatment exists. We report the results of high-dose therapy and autologous stem cell transplantation (ASCT) or reduced-intensity conditioning and allogeneic stem cell transplantation (alloSCT) in patients with multiply relapsed LG. A European Group for Blood and Marrow Transplantation survey identified 10 patients who had received 9 ASCT and 4 alloSCT. All patients had active disease at the time of transplantation. With a median follow-up of 5.1 (range, 1.4 to 6.3) years, 6 patients are alive and disease-free. Two ASCT patients died of septicemia early after transplantation, and 1 committed suicide after being in continuous complete remission 19 months after ASCT. Another patient allografted 4 years after ASCT remained disease-free but died of severe graft-versus-host disease 3 months after alloSCT. High-dose therapy followed by ASCT and alloSCT are effective therapeutic options and should be considered in all patients with refractory and multiply relapsed LG.
AuthorsKristina Siegloch, Norbert Schmitz, Huei-Shan Wu, Birte Friedrichs, Gustaaf W van Imhoff, Silvia Montoto, Ernst Holler, Josep Maria Ribera, Robert Delage, Ulrich Dührsen, Nerea Castillo, Nerea Del Castillo, Beth Harrison, Peter Dreger, Anna Sureda, Working Party Lymphoma of the European Group for Blood and Marrow Transplantation (EBMT)
JournalBiology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation (Biol Blood Marrow Transplant) Vol. 19 Issue 10 Pg. 1522-5 (Oct 2013) ISSN: 1523-6536 [Electronic] United States
PMID23948061 (Publication Type: Journal Article, Multicenter Study)
CopyrightCopyright © 2013 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.
Topics
  • Adult
  • Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
  • Disease-Free Survival
  • Europe
  • Female
  • Hematopoietic Stem Cell Transplantation (methods)
  • Humans
  • Lymphomatoid Granulomatosis (drug therapy, surgery)
  • Male
  • Middle Aged
  • Transplantation Conditioning (methods)
  • Transplantation, Homologous
  • Young Adult

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