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Allogeneic stem cell transplantation for the treatment of refractory scleromyxedema.

Abstract
Scleromyxedema is a rare disorder of connective tissue with unknown etiology. Its manifestation includes a generalized mucin deposition, which is frequently associated with paraproteinemia. The course of scleromyxedema is progressive and often lethal. As a result of its poorly understood pathogenesis, there is no causative treatment option. The efficacy of cytoreductive agents and autologous stem cell transplantation has been reported, but so far allografting as a treatment option has not yet been documented. Herein, we report on a patient with severe neurologic involvement and refractory course attaining durable remission after receiving an allogeneic hematopoietic cell transplant from an human leukocyte antigen-matched sibling. This case not only illustrates a potential new treatment option for selected patients, but also provides insights into the pathogenesis of this rare disease.
AuthorsNona Shayegi, Nael Alakel, Jan Moritz Middeke, J Schetelig, Luisa Mantovani-Löffler, Martin Bornhäuser
JournalTranslational research : the journal of laboratory and clinical medicine (Transl Res) Vol. 165 Issue 2 Pg. 321-4 (Feb 2015) ISSN: 1878-1810 [Electronic] United States
PMID24999269 (Publication Type: Case Reports, Journal Article)
CopyrightCopyright © 2015 Elsevier Inc. All rights reserved.
Topics
  • Adult
  • Allografts
  • Central Nervous System Diseases (etiology, immunology, therapy)
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Male
  • Paraproteinemias (complications, immunology, therapy)
  • Remission Induction
  • Scleromyxedema (complications, immunology, therapy)
  • Translational Research, Biomedical

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