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

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 reduce...

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Main Authors: Siegloch, Kristina (Author) , Schmitz, Norbert (Author) , Wu, Huei-Shan (Author) , Friedrichs, Birte (Author) , van Imhoff, Gustaaf W. (Author) , Montoto, Silvia (Author) , Holler, Ernst (Author) , Ribera, Josep Maria (Author) , Delage, Robert (Author) , Dührsen, Ulrich (Author) , Castillo, Nerea del (Author) , Harrison, Beth (Author) , Dreger, Peter (Author) , Sureda, Anna (Author)
Format: Article (Journal)
Language:English
Published: 2013
In: Biology of blood and marrow transplantation
Year: 2013, Volume: 19, Issue: 10, Pages: 1522-1525
ISSN:1523-6536
DOI:10.1016/j.bbmt.2013.07.023
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.bbmt.2013.07.023
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S1083879113003455
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Author Notes:Kristina Siegloch, Norbert Schmitz, Huei-Shan Wu, Birte Friedrichs, Gustaaf W. van Imhoff, Silvia Montoto, Ernst Holler, Josep Maria Ribera, Robert Delage, Ulrich Dührsen, Nerea del Castillo, Beth Harrison, Peter Dreger, Anna Sureda, on behalf of theWorking Party Lymphoma of the European Group for Blood and Marrow Transplantation (EBMT)

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520 |a 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. 
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