title: [Immunosuppression with cyclophosphamide and fudarabine, with support of autologous CD-34+ stem cells, in the treatment of severe scleroderma].
abstract: Immune ablation of T lymphocytes, key players in the physiopathogenic processes leading to autoimmune diseases, may arrest scleroderma progression even in some severely ill patients. High-dose chemotherapy followed by autologous stem cell reinfusion - sometimes named peripheral blood stem cell transplantation - has been an effective therapy for some autoimmune diseases, including scleroderma. In this paper the authors present a patient with the diffuse form of scleroderma who received immunosuppression with cyclophosphamide 0.5 g/m² and fludarabine followed by infusion of autologous CD-34+ cells without T lymphocyte purging. This patient was followed up with cutaneous score measures, imaging and laboratory tests, as well as a well-being questionnaire. After transplantation there were major positive responses on skin and gastrointestinal tract, with cessation of diarrhea and malabsorption syndrome. These clinical improvements did not last past 6 months. We observed transitory stem cell transfusion reaction and localized herpes zoster. The patient died 12 months after the procedure due to aspirative pneumonia and gastrointestinal complications. The immunosuppressive regimen, and not the autologous stem cell transplantation, could have been responsible for the observed transitory patient improvement.
authors.affiliation: Faculdade de Medicina, PUCRS.
authors.forename: Carlos Alberto von
authors.initials: CA
authors.lastname: Mühlen
authors.forename: Everton Luís
authors.initials: EL
authors.lastname: Hinterholz
authors.forename: Bernardo
authors.initials: B
authors.lastname: Garicochea
authors.forename: Pedro
authors.initials: P
authors.lastname: Weingrill
authors.forename: Carlos
authors.initials: C
authors.lastname: Barrios
authors.forename: Mário Sérgio
authors.initials: MS
authors.lastname: Fernandes
authors.forename: Rosângela
authors.initials: R
authors.lastname: Dahmer
authors.forename: Mauro W
authors.initials: MW
authors.lastname: Keiserman
authors.forename: Henrique Luiz
authors.initials: HL
authors.lastname: Staub