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Schnitzler syndrome associated with systemic marginal zone B‐cell lymphoma
Author(s) -
Dalle S.,
Balme B.,
Sebban C.,
Pariset C.,
Berger F.,
Thomas L.
Publication year - 2006
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2006.07417.x
Subject(s) - marginal zone , medicine , lymphoma , dermatology , pathology , immunology , b cell , antibody
Summary 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.