Schnitzler syndrome associated with systemic marginal zone B-cell lymphoma.
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.
Dalle
S
S
Service d'Hématologie, Centre Léon Bérard, Lyon, France. stephane.dalle@chu-lyon.fr
Balme
B
B
Sebban
C
C
Pariset
C
C
Berger
F
F
Thomas
L
L
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*
D007075:Immunoglobulin M