Document Detail


Polyarteritis nodosa complicating multiple myeloma - a case report and review of the literature.
MedLine Citation:
PMID:  24220008     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Introduction: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of smallto-medium-sized vessels, rarely associated with hematologic neoplasms. Case report: We report a 44-year-old man with a history of monoclonal gammopathy of undetermined significance (MGUS) who presented with rapidly progressing sensorimotor peripheral neuropathy. Two weeks after onset the patient developed severe acute acral and retinal ischemia. MR-angiography and nerve biopsy revealed a systemic necrotizing vasculitis (PAN type). At this time, bone marrow biopsy identified a smoldering multiple myeloma. Immediate immunosuppressive and anti-neoplastic treatment (steroids, immunoglobulins, bortezomib combined with cyclophosphamide followed by lenalidomide maintenance) resulted in a favorable clinical outcome. After 4 years, the patient is in good clinical condition with sustained partial remission from myeloma and without evidence of relapse of PAN. Conclusion: This is a remarkable case of a histologically confirmed peripheral neuropathy due to polyarteritis nodosa associated with progression of MGUS to multiple myeloma. Immediate diagnosis and combined immunosuppressive and anti-neoplastic treatment may improve the outcome of this potentially life-threatening clinical condition.
Authors:
Markus Hutterer; Michael Steurer; Romana Höftberger; Johannes A Hainfellner; Andreas Pircher; Martha Nowosielski; Peter Hau; Wolfgang Löscher; Eberhard Gunsilius; Thomas Berger; Guenther Stockhammer
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-11-13
Journal Detail:
Title:  Clinical neuropathology     Volume:  -     ISSN:  0722-5091     ISO Abbreviation:  Clin. Neuropathol.     Publication Date:  2013 Nov 
Date Detail:
Created Date:  2013-11-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8214420     Medline TA:  Clin Neuropathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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