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New onset of myasthenia gravis after treatment of systemic sclerosis by autologous hematopoietic stem cell transplantation: sustained autoimmunity or inadequate reset of tolerance?

Abstract
Autologous hematopoietic stem-cell transplantation (HSCT) showed promising results for the treatment of primary severe autoimmune diseases (ADs). In this context, development of secondary AD after HSCT has exceptionally been observed, further questioning the roles of patient propensity for AD and of the HSCT procedure. Herein, we report new onset of myasthenia gravis 3 years after successful HSCT in a patient with severe systemic sclerosis, while in complete remission from her first AD. The de novo occurrence of secondary AD (myasthenia gravis) after HSCT was accompanied by the appearance of clonal T-cell expansions measured by the "immunoscope" technique in the context of an ongoing T-cell immune reconstitution. Secondary ADs are increasingly recognized after HSCT for AD. In our case, development of myasthenia followed clonal T-cell expansion. Detailed T-cell repertoire analysis may shed light on autoreactivity mechanisms after HSCT and may help to identify patients at risk.
AuthorsChristophe Deligny, Emmanuel Clave, David Sibon, Thomas Daikeler, Homah Keshmandt, Maryvonnic Carmagnat, Corinne Douay, Serge Arfi, Bernard Clair, Antoine Toubert, Dominique Farge
JournalHuman immunology (Hum Immunol) Vol. 71 Issue 4 Pg. 363-5 (Apr 2010) ISSN: 1879-1166 [Electronic] United States
PMID20085795 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Receptors, Antigen, T-Cell
  • DNA
Topics
  • Autoimmunity
  • Cell Proliferation
  • Cells, Cultured
  • DNA (analysis)
  • Female
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Immune Tolerance
  • Middle Aged
  • Myasthenia Gravis (diagnosis, etiology, genetics, physiopathology)
  • Pathology, Molecular
  • Postoperative Complications
  • Receptors, Antigen, T-Cell (biosynthesis, genetics, immunology)
  • Remission Induction
  • Scleroderma, Systemic (complications, therapy)
  • T-Lymphocytes (immunology, metabolism, pathology)
  • Transplantation, Autologous

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