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Blood, 15 January 2006, Vol. 107, No. 2, pp. 463-466.
Prepublished online as a Blood First Edition Paper on September 22, 2005; DOI 10.1182/blood-2004-12-4870.
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Submitted January 5, 2005
Accepted August 22, 2005
Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation
Michele L Donato, Adrienne M Feasel, Donna M Weber, Victor G Prieto, Sergio A Giralt, Richard E Champlin, and Madeleine Duvic*
Department of Blood and Bone Marrow Transplantation, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA
Department of Dermatology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA
Department of Myeloma, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA
Department of Dermatopathology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA
* Corresponding author; email: mduvic{at}mdanderson.org.
Scleromyxedema, the most severe manifestation of the spectrum of lichen myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of eight patients evaluated at our center were treated with high-dose melphalan 180 mg/m2 IV 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 two patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months post-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 transplant appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

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