A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient

Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients its etiology is still unknown, hence the designation of idiopathic multicentric Castleman disease (iMCD). To successful...

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Autores principales:	Luís Landeiro, Ana Carolina Freitas, Margarida Proença, José Cabeçadas, Albertina Nunes, Alexandra Bayão Horta
Formato:	article
Lenguaje:	EN
Publicado:	SMC MEDIA SRL 2021
Materias:	multicentric castleman nephrotic syndrome polyneuropathy Medicine R
Acceso en línea:	https://doaj.org/article/89eee749381647aea23497852fc5c0d2
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id	oai:doaj.org-article:89eee749381647aea23497852fc5c0d2
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spelling	oai:doaj.org-article:89eee749381647aea23497852fc5c0d22021-11-09T11:21:26ZA Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient2284-259410.12890/2021_002831https://doaj.org/article/89eee749381647aea23497852fc5c0d22021-10-01T00:00:00Zhttps://www.ejcrim.com/index.php/EJCRIM/article/view/2831https://doaj.org/toc/2284-2594Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients its etiology is still unknown, hence the designation of idiopathic multicentric Castleman disease (iMCD). To successfully diagnose iMCD, diagnostic criteria must be fulfilled and a large array of alternative diagnosis 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 laboratorial remission was achieved and maintained at 2-year follow-up.Luís LandeiroAna Carolina FreitasMargarida ProençaJosé CabeçadasAlbertina NunesAlexandra Bayão HortaSMC MEDIA SRLarticlemulticentric castlemannephrotic syndromepolyneuropathyMedicineRENEuropean Journal of Case Reports in Internal Medicine (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	multicentric castleman nephrotic syndrome polyneuropathy Medicine R
spellingShingle	multicentric castleman nephrotic syndrome polyneuropathy Medicine R Luís Landeiro Ana Carolina Freitas Margarida Proença José Cabeçadas Albertina Nunes Alexandra Bayão Horta A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient
description	Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients its etiology is still unknown, hence the designation of idiopathic multicentric Castleman disease (iMCD). To successfully diagnose iMCD, diagnostic criteria must be fulfilled and a large array of alternative diagnosis 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 laboratorial remission was achieved and maintained at 2-year follow-up.
format	article
author	Luís Landeiro Ana Carolina Freitas Margarida Proença José Cabeçadas Albertina Nunes Alexandra Bayão Horta
author_facet	Luís Landeiro Ana Carolina Freitas Margarida Proença José Cabeçadas Albertina Nunes Alexandra Bayão Horta
author_sort	Luís Landeiro
title	A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient
title_short	A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient
title_full	A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient
title_fullStr	A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient
title_full_unstemmed	A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient
title_sort	rare association between idiopathic multicentric castleman disease, nephrotic syndrome and polyneuropathy in an immunocompetent patient
publisher	SMC MEDIA SRL
publishDate	2021
url	https://doaj.org/article/89eee749381647aea23497852fc5c0d2
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A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient

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