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Allogeneic hematopoietic stem cell transplantation for advanced mycosis fungoides and Sézary syndrome. An updated experience of the Lymphoma Working Party of the European Society for Blood and Marrow Transplantation

Bone Marrow Transplantation volume 56, pages 1391–1401 (2021)Cite this article

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Abstract

Background

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a potentially curative treatment option in advanced-stage mycosis fungoides (MF) and Sézary syndrome (SS). This study presents an updated analysis of the initial experience of the Lymphoma Working Party of the European Society for Blood and Marrow Transplantation (EBMT) describing the outcomes after allo-HSCT for MF and SS, with special emphasis on the impact of the use of unrelated donors (URD).

Methods and patients

Eligible for this study were patients with advanced-stage MF or SS who underwent a first allo-HSCT from matched HLA-identical related or URD between January/1997 and December/2011. Sixty patients have been previously reported.

Results

113 patients were included [77 MF (68%)]; 61 (54%) were in complete or partial remission, 86 (76%) received reduced-intensity protocols and 44 (39%) an URD allo-HSCT. With a median follow up for surviving patients of 73 months, allo-HSCT resulted in an estimated overall survival (OS) of 38% at 5 years, and a progression-free survival (PFS) of 26% at 5 years. Multivariate analysis demonstrated that advanced-phase disease (complete remission/partial remission >3, primary refractory or relapse/progression in patients that had received 3 or more lines of systemic treatment prior to transplant or the number of treatment lines was not known), a short interval between diagnosis and transplant (<18 months) were independent adverse prognostic factors for PFS; advanced-phase disease and the use of URDs were independent adverse prognostic factors for OS.

Conclusions

This extended series supports that allo-HSCT is able to effectively rescue over one third of the population of patients with advanced-stage MF/SS. High relapse rate is still the major cause of failure and needs to be improved with better strategies before and after transplant. The negative impact of URD is a matter of concern and needs to be further elucidated in future studies.

Highlights

  • Allogeneic transplantation is a potentially curative option for advanced-stage mycosis fungoides and Sézary syndrome.

  • Advanced-phase disease constitutes the most important prognostic factor for the long term outcome of the patients.

  • The negative impact of the use of unrelated donors needs to be further elucidated.

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Fig. 1
Fig. 2: Long term outcomes of the global series.
Fig. 3: Impact of the disease phase at the time of allo-HSCT in progression-free survival and overall survival.
Fig. 4: Impact of the type of donor in progression-free survival and overall survival.

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Acknowledgements

The authors acknowledge all collaborating EBMT Investigators and Institutions that contributed cases to this study.

Author information

Author notes

  1. These authors contributed equally: E. Domingo-Domenech and R.F. Duarte

Authors and Affiliations

  1. Hematology Department, Institut Català d’Oncologia. Hospital Duran i Reynals, IDIBELL, Universitat de Barcelona, Barcelona, Spain

    E. Domingo-Domenech & A. Sureda

  2. Hematology Department, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, Spain

    R. F. Duarte

  3. EBMT Central Registry Office, Paris, France

    A. Boumedil & H. Finel

  4. Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico IRCCS, Milano, Italy

    F. Onida & A. Cortelezzi

  5. Department of Hematology, Imperial College, Hammersmith Hospital, London, United Kingdom

    I. Gabriel & E. Olavarria

  6. Tor Vergata University of Rome, Stem Cell Transplant Unit, Policlinico Universitario Tor Vergata, Rome, Italy

    W. Arcese

  7. St’s James Hospital, Dublin, Ireland

    P. Browne

  8. University Hospital, Department of Bone Marrow Transplantation, Essen, Germany

    D. Beelen

  9. Heinrich Heine University, Medical F, Department of Hematology, Düsseldorf, Germany

    G. Kobbe

  10. Leiden University Medical Center, Leiden, The Netherlands

    H. Veelken

  11. Hematology Department, Hospital La Princesa, Madrid, Spain

    R. Arranz

  12. Division of Hematology, Medical University Graz, Graz, Austria

    H. Greinix

  13. Skanes University Hospital, Department of Hematology, Lund, Sweden

    S. Lenhoff

  14. Cliniques Universitaires St. Luc, Department of Hematology, Brussels, Belgium

    X. Poiré

  15. Hematology Department, Institut Català d’Oncologia, Josep Carreras Leukemia Research Institute, Badalona, Spain

    J. M. Ribera

  16. Albert’s Stem Cell Transplantation Center, Pretoria, South Africa

    J. Thompson

  17. Ramban Medical Center, Department of Hematology and Bone Marrow Transplantation, Haifa, Israel

    T. Zuckerman

  18. GKT School of Medicine, Dept. of Haematological Medicine, King’s Denmark Hill Campus, London, United Kingdom

    G. J. Mufti

  19. Universitaetsklinkum Heidelberg, Heidelberg, Germany

    P. Dreger

  20. Department of Haemato-Oncology, St. Bartholomew’s Hospital, Barts Health NHS Trust, London, United Kingdom

    S. Montoto

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  1. E. Domingo-Domenech
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Correspondence to E. Domingo-Domenech.

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Domingo-Domenech, E., Duarte, R.F., Boumedil, A. et al. Allogeneic hematopoietic stem cell transplantation for advanced mycosis fungoides and Sézary syndrome. An updated experience of the Lymphoma Working Party of the European Society for Blood and Marrow Transplantation. Bone Marrow Transplant 56, 1391–1401 (2021). https://doi.org/10.1038/s41409-020-01197-3

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