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.
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Authors | Nona Shayegi, Nael Alakel, Jan Moritz Middeke, J Schetelig, Luisa Mantovani-Löffler, Martin Bornhäuser |
Journal | Translational 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 |
PMID | 24999269
(Publication Type: Case Reports, Journal Article)
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Copyright | Copyright © 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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