title: Schnitzler syndrome associated with systemic marginal zone B-cell lymphoma.
abstract: Schnitzler syndrome is a rare condition defined by chronic urticaria and monoclonal IgM gammopathy. Malignant evolution to Waldenström disease (WD) has been reported in several cases. We report a 49-year-old man who developed a marginal zone B-cell lymphoma (MZL) 3 years after the beginning of Schnitzler syndrome. This is the first report of MZL in association with Schnitzler syndrome. IgM gammopathy is a clue for the diagnosis of Schnitzler syndrome. This condition needs to be closely monitored; it can precede the onset of an authentic lymphoproliferative disorder including WD and rarely MZL.
authors.affiliation: Service d'Hématologie, Centre Léon Bérard, Lyon, France. stephane.dalle@chu-lyon.fr
authors.forename: S
authors.initials: S
authors.lastname: Dalle
authors.forename: B
authors.initials: B
authors.lastname: Balme
authors.forename: C
authors.initials: C
authors.lastname: Sebban
authors.forename: C
authors.initials: C
authors.lastname: Pariset
authors.forename: F
authors.initials: F
authors.lastname: Berger
authors.forename: L
authors.initials: L
authors.lastname: Thomas
mesh_terms: D002471:Cell Transformation, Neoplastic / Q000473:pathology; D006801:Humans; D007075:Immunoglobulin M; D008198:Lymph Nodes / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D019873:Schnitzler Syndrome / Q000175:diagnosis / Q000473:pathology*
chemical_list: D007075:Immunoglobulin M