Hutterer, Markus and Steurer, Michael and Hoeftberger, Romana and Hainfellner, Johannes A. and Pircher, Andreas and Nowosielski, Martha and Hau, Peter and Loescher, Wolfgang and Gunsilius, Eberhard and Bergerl, Thomas and Stockhammer, Guenther (2014) Polyarteritis nodosa complicating multiple myeloma - a case report and review of the literature. CLINICAL NEUROPATHOLOGY, 33 (2). pp. 143-151. ISSN 0722-5091,
Full text not available from this repository. (Request a copy)Abstract
Introduction: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of small-to-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, borte-zomib 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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | UNDETERMINED SIGNIFICANCE; MONOCLONAL GAMMOPATHY; PERIPHERAL NEUROPATHY; SYSTEMIC VASCULITIS; NECROTIZING VASCULITIS; CLINICAL-FEATURES; STAGING SYSTEM; POEMS SYNDROME; MANAGEMENT; DIAGNOSIS; MGUS; multiple myeloma; vasculitis; polyarteritis nodosa; peripheral polyneuropathy |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 18 Nov 2019 15:22 |
| Last Modified: | 18 Nov 2019 15:22 |
| URI: | https://pred.uni-regensburg.de/id/eprint/10553 |
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