Refractory Evans' syndrome treated with allogeneic SCT followed by DLI. Demonstration of a graft-versus-autoimmunity effect

A. M. Marmont, F. Gualandi, M. T. van Lint, A. Bacigalupo

Research output: Contribution to journalArticle

Abstract

Evans' syndrome, a combination of autoimmune haemolytic anaemia and autoimmune (idiopathic) thrombocytopenic purpura, is generally harder to treat and more refractory than the single entities. In a male patient with refractory disease, predominantly thrombocytopenic, an allogeneic reduced intensity BMT from his human leukocyte antigen (HLA)-identical sister was followed by a dramatic platelet peak while he was still experiencing initial engraftment (presumably of autologous origin), but subsequently by progressive relapse associated with mixed chimerism. Five gradually incremental DLI achieved complete donor chimerism, which was associated not only with grade II graft-versus-host disease (GVHD), but also with complete clinical and biological remission for 2 years post-transplant. Long-term FU is necessary before claiming that allogeneic stem cell transplantation (SCT) is capable of curing an autoimmune blood disease. However, there is evidence for a graft-versus-autoimmunity effect in this case.

Original languageEnglish
Pages (from-to)399-402
Number of pages4
JournalBone Marrow Transplantation
Volume31
Issue number5
DOIs
Publication statusPublished - Mar 2003

Fingerprint

Chimerism
Idiopathic Thrombocytopenic Purpura
Stem Cell Transplantation
Autoimmunity
Transplants
Autoimmune Hemolytic Anemia
Hematologic Diseases
Graft vs Host Disease
HLA Antigens
Autoimmune Diseases
Siblings
Blood Platelets
Tissue Donors
Recurrence
Evans Syndrome

Keywords

  • Allogeneic STC
  • Evans syndrome
  • Graft-versus-autoimmunity

ASJC Scopus subject areas

  • Hematology
  • Transplantation

Cite this

Refractory Evans' syndrome treated with allogeneic SCT followed by DLI. Demonstration of a graft-versus-autoimmunity effect. / Marmont, A. M.; Gualandi, F.; van Lint, M. T.; Bacigalupo, A.

In: Bone Marrow Transplantation, Vol. 31, No. 5, 03.2003, p. 399-402.

Research output: Contribution to journalArticle

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