PMID: 9433380Jan 20, 1998Paper

Mucocutaneous autoimmune syndrome following fludarabine therapy for low-grade non-Hodgkin's lymphoma of B-cell type (B-NHL)

Annals of Hematology
J BraessB Wörmann

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.

Citations

Apr 30, 2003·European Journal of Internal Medicine·Brigitte GranelPierre Jean Weiller
Jan 25, 2005·Clinical and Experimental Dermatology·G K PereraD Creamer
May 10, 2007·Leukemia & Lymphoma·Ewa Robak, Tadeusz Robak
Jul 6, 2005·La Revue de médecine interne·F JardinH Tilly
Mar 18, 2008·Journal of the American Academy of Dermatology·Noushin HeidarySusan Burgin
Mar 1, 2008·Expert Review of Clinical Immunology·Fabrice Jardin
May 1, 2008·Expert Review of Clinical Immunology·Pamela VezzoliAngelo V Marzano
Jan 9, 2021·International Immunopharmacology·Forugh GhaediMaryam Daneshpazhooh

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