Landeiro, Luís and Freitas, Ana Carolina and Proença, Margarida and Cabeçadas, José and Nunes, Albertina and Bayão Horta, Alexandra (2021) Rare Association Between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an Immunocompetent Patient. European Journal of Case Reports in Internal Medicine, 8 (10). pp. 1-4. ISSN 2284-2594
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Abstract
Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients the aetiology is still unknown, hence the designation of idiopathic MCD (iMCD). To successfully diagnose iMCD, diagnostic criteria must be fulfilled and a large array of alternative diagnoses excluded. Peripheral neuropathy and nephropathy are relatively common findings in cases associated with POEMS syndrome, but very rarely reported in iMCD.We present the case of a 64-year-old man with iMCD (HIV- and HHV-8-negative) with nephrotic syndrome and severe motor polyneuropathy. Alternative diagnoses were excluded. The patient was treated with intravenous glucocorticoid followed by rituximab. Complete clinical and laboratory remission was achieved and maintained at the 2-year follow-up.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | Multicentric Castleman, nephrotic syndrome, polyneuropathy |
| Subjects: | 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) |
| Depositing User: | Marina Spanti |
| Date Deposited: | 17 Feb 2022 12:04 |
| Last Modified: | 17 Feb 2022 12:04 |
| URI: | http://eprints.bice.rm.cnr.it/id/eprint/21723 |
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