Durable remission of Sézary syndrome after unrelated bone marrow transplantation by reduced-intensity conditioning

Acta Haematol. 2008;120(1):14-8. doi: 10.1159/000151510. Epub 2008 Aug 21.

Abstract

A 22-year-old Japanese man was diagnosed with Sézary syndrome with large cell transformation. His skin lesions persisted after treatment with 7 cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone), psoralen and ultraviolet light A, and total skin electron beam irradiation. He subsequently underwent allogeneic bone marrow transplantation by reduced-intensity conditioning from a human leukocyte antigen-identical unrelated donor. He developed grade II of acute graft-versus-host disease and extensive-type chronic graft-versus-host disease. He has no signs of disease 36 months after the transplantation. The prognosis of patients with advanced stage of mycosis fungoides or Sézary syndrome is very poor. Allogeneic hematopoietic stem cell transplantation, especially by reduced-intensity conditioning, is expected to become a curative treatment option, and graft-versus-tumor effect might play a critical role for sustained remission.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols
  • Bone Marrow Transplantation*
  • Cyclophosphamide
  • Doxorubicin
  • Graft vs Host Disease
  • Graft vs Tumor Effect
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Male
  • Prednisone
  • Remission Induction
  • Sezary Syndrome / drug therapy
  • Sezary Syndrome / pathology
  • Sezary Syndrome / therapy*
  • Transplantation Conditioning*
  • Transplantation, Homologous
  • Vincristine

Substances

  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • Prednisone

Supplementary concepts

  • CHOP protocol