Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation. Academic Article uri icon

Overview

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.

publication date

  • September 22, 2005

Research

keywords

  • Antineoplastic Agents, Alkylating
  • Hematopoietic Stem Cell Transplantation
  • Melphalan
  • Myxedema
  • Scleroderma, Limited
  • Scleroderma, Systemic

Identity

PubMed Central ID

  • PMC1895605

Scopus Document Identifier

  • 30444439102

Digital Object Identifier (DOI)

  • 10.1182/blood-2004-12-4870

PubMed ID

  • 16179379

Additional Document Info

volume

  • 107

issue

  • 2