Malignant Evolution of Schnitzler Syndrome to Waldenström Macroglobulinemia: a Case Report

Abstract

Introduction: Schnitzler syndrome (SchS), first described in 1972, is a rare autoinflammatory condition characterized by chronic urticaria and monoclonal gammopathy of IgM or, exceptionally, IgG profile. Additional features include recurrent fever, evidence of abnormal bone remodeling, a neutrophilic dermal infiltrate on skin biopsy, leukocytosis or elevated C-reactive protein, according to the Strasbourg criteria. Case Presentation: We describe the case of a 56-year-old Caucasian male patient, who suffered from chronic urticaria, moderate-grade fever, severe generalized fatigue and arthralgias. After five years of chronic disease evolution, he was referred to the hematology department where he was found to have IgM kappa light chain monoclonal gammopathy. The constellation of symptoms, a negative rheumatologic workup, and the finding of IgM monoclonal gammopathy determined the diagnosis of Schnitzler syndrome. Bone marrow biopsy proved the association of Waldenström macroglobulinemia. Conclusion: The main goal of our case report was to highlight the clinical features and treatment, with emphasis on the hematological aspects, to provide a better understanding and to raise awareness of Schnitzler syndrome among healthcare professionals.