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Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation.

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.
AuthorsMichele L Donato, Adrienne M Feasel, Donna M Weber, Victor G Prieto, Sergio A Giralt, Richard E Champlin, Madeleine Duvic
JournalBlood (Blood) Vol. 107 Issue 2 Pg. 463-6 (Jan 15 2006) ISSN: 0006-4971 [Print] United States
PMID16179379 (Publication Type: Journal Article, Research Support, N.I.H., Extramural)
Chemical References
  • Antineoplastic Agents, Alkylating
  • Melphalan
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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