| Malignant evolution of Schnitzler's syndrome--chronic urticaria and IgM monoclonal gammopathy: report of a new case and review of the literature. | |
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MedLine Citation:
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PMID: 11908725 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Schnitzler's syndrome, initially described in 1974 is an uncommon condition defined by chronic urticaria and monoclonal IgM gammopathy. Additional features include fever of unknown origin, elevated ESR, bone pain and frequently a benign clinical course. We conducted a literature search of Medline, EMBASE and Cancerlit and found 56 cases of Schnitzler's syndrome reported to date. The absence of lymphoproliferative disease in this condition is typical, but nine patients have progressed to develop lymphoplasmacytic neoplasias, particularly Waldenstrom's macroglobulinemia (WM). Malignant evolution of Schnitzler's syndrome is a rare complication, but emphasizes the importance of long term follow-up and the need for these patients to undergo periodic assessment of the bone marrow and lymph nodes. Treatment of this condition is difficult, with varying response to corticosteroids and largely unsuccessful results with standard chemotherapy used for WM. We describe a case of Schnitzler's syndrome in a 50-year old man with lymphocytic aggregates in the bone marrow after 9 years of chronic urticaria, fever, arthralgias and bone pain. We review the clinical features and treatment, with emphasis on the hematologic aspects of this unusual condition. |
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Authors:
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W Lim; K H Shumak; M Reis; B Perez-Ordonez; D Sauder; A Fam; K R Imrie |
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Publication Detail:
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Type: Case Reports; Journal Article; Review |
Journal Detail:
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Title: Leukemia & lymphoma Volume: 43 ISSN: 1042-8194 ISO Abbreviation: Leuk. Lymphoma Publication Date: 2002 Jan |
Date Detail:
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Created Date: 2002-03-22 Completed Date: 2003-07-29 Revised Date: 2005-11-16 |
Medline Journal Info:
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Nlm Unique ID: 9007422 Medline TA: Leuk Lymphoma Country: Switzerland |
Other Details:
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Languages: eng Pagination: 181-6 Citation Subset: IM |
Affiliation:
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University of Toronto, Ont., Canada. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Bone Marrow
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pathology Cell Transformation, Neoplastic / pathology* Chronic Disease Humans Immunoglobulin M Lymphocytes / pathology Male Middle Aged Paraproteinemias / etiology Schnitzler Syndrome / pathology* Urticaria Waldenstrom Macroglobulinemia / etiology |
| Chemical | |
Reg. No./Substance:
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0/Immunoglobulin M |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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