title: Mucocutaneous autoimmune syndrome following fludarabine therapy for low-grade non-Hodgkin's lymphoma of B-cell type (B-NHL).
abstract: A 40-year-old patient with low-grade B-NHL developed a generalized macular-papular rash following the first cycle of fludarabine treatment which progressed to a complete epidermal necrolysis following the second cycle. Clinical symptoms and the results of the direct and indirect immunofluorescence were consistent with a mucocutaneous autoimmune syndrome (pemphigus). Immunohistochemical analysis demonstrated a dense epidermal infiltration of CD8+ lymphocytes associated with the histological features of single-cell necrosis of keratinocytes. Early and aggressive immunosuppressive treatment with steroids, cyclophosphamide, and high-dose immunoglobulins resulted in regression of symptoms and complete reconstitution of epidermal integrity. The malignant lymphoma has completely regressed. The findings suggest a fludarabine-induced defect in immunosurveillance--resulting in the uncontrolled activation of autoaggressive T-cell clones--as a pathogenetic mechanism of this life-threatening dermatological complication.
authors.affiliation: Department of Hematology and Oncology, Klinikum Göttingen, Georg August Universität, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Braess
authors.forename: K
authors.initials: K
authors.lastname: Reich
authors.forename: S
authors.initials: S
authors.lastname: Willert
authors.forename: F
authors.initials: F
authors.lastname: Strutz
authors.forename: C
authors.initials: C
authors.lastname: Neumann
authors.forename: W
authors.initials: W
authors.lastname: Hiddemann
authors.forename: B
authors.initials: B
authors.lastname: Wörmann
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000009:adverse effects* / Q000627:therapeutic use; D015551:Autoimmunity; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects* / Q000627:therapeutic use; D016393:Lymphoma, B-Cell / Q000188:drug therapy*; D008297:Male; D012871:Skin Diseases / Q000139:chemically induced* / Q000276:immunology; D013577:Syndrome; D014740:Vidarabine / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D014740:Vidarabine; C024352:fludarabine