Abstract |
Scleromyxedema, the most severe manifestation of the spectrum of lichen myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m(2) intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The lichen myxedematosus- scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.
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Authors | Michele L Donato, Adrienne M Feasel, Donna M Weber, Victor G Prieto, Sergio A Giralt, Richard E Champlin, Madeleine Duvic |
Journal | Blood
(Blood)
Vol. 107
Issue 2
Pg. 463-6
(Jan 15 2006)
ISSN: 0006-4971 [Print] United States |
PMID | 16179379
(Publication Type: Journal Article, Research Support, N.I.H., Extramural)
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Chemical References |
- Antineoplastic Agents, Alkylating
- Melphalan
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Topics |
- Adult
- Aged
- Antineoplastic Agents, Alkylating
(administration & dosage)
- Dose-Response Relationship, Drug
- Female
- Hematopoietic Stem Cell Transplantation
- Humans
- Male
- Maximum Tolerated Dose
- Melphalan
(administration & dosage)
- Middle Aged
- Myxedema
(pathology, therapy)
- Palliative Care
- Remission Induction
- Scleroderma, Limited
(pathology, therapy)
- Scleroderma, Systemic
(pathology, therapy)
- Transplantation, Autologous
- Treatment Outcome
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