<abstract xmlns="http://www.w3.org/1999/xhtml">

<bold>Introduction:</bold> 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.
<bold>Case Presentation:</bold> 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.
<bold>Conclusion:</bold> 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.
</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.

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

Department of Morphopathology, Emergency County Clinical Hospital

“George Emil Palade” University of Medicine, Pharmacy, Science and Technology

Journal of Interdisciplinary Medicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Introduction: Schnitzler syndrome (SchS), first described in 1972, is a rare autoinflammatory condition characterized by chronic urticaria and monoclonal...

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

Published Online: Nov 07, 2022

Page range: 74 - 77

Received: Sep 01, 2022

Accepted: Sep 05, 2022

DOI: https://doi.org/10.2478/jim-2022-0011

Keywords
Schnitzler syndrome, Waldenström macroglobulinemia, IgM monoclonal gammopathy, autoinflammatory disease, chronic urticaria

© 2022 Cristina-Alexandra Man et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

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

Published Online: Nov 07, 2022

Page range: 74 - 77

Received: Sep 01, 2022

Accepted: Sep 05, 2022

DOI: https://doi.org/10.2478/jim-2022-0011

KeywordsSchnitzler syndrome, Waldenström macroglobulinemia, IgM monoclonal gammopathy, autoinflammatory disease, chronic urticaria

© 2022 Cristina-Alexandra Man et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Keywords
Schnitzler syndrome, Waldenström macroglobulinemia, IgM monoclonal gammopathy, autoinflammatory disease, chronic urticaria