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Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association

Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal pla...

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Autores principales: Belabbes, Fatima, Houda, Youssefi, Al Bouzidi, Abderahmane, Bennani, Youssef, Ahnach, Maryame
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9187135/
https://www.ncbi.nlm.nih.gov/pubmed/35698702
http://dx.doi.org/10.7759/cureus.24923
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author Belabbes, Fatima
Houda, Youssefi
Al Bouzidi, Abderahmane
Bennani, Youssef
Ahnach, Maryame
author_facet Belabbes, Fatima
Houda, Youssefi
Al Bouzidi, Abderahmane
Bennani, Youssef
Ahnach, Maryame
author_sort Belabbes, Fatima
collection PubMed
description Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma (PCD), and at least one of the minor criteria. The most frequent liver manifestation is hepatomegaly. Idiopathic portal hypertension (IPH) has been reported rarely in POEMS syndrome. The precise etiopathogenesis of IPH is not fully elucidated. We report a 46-year-old male patient presenting with POEMS syndrome. He presented postprandial vomiting and left tinnitus. Loss of appetite, leanness, and excessive sweat were concomitant symptoms. Abdominal examination revealed splenomegaly and an absence of hepatomegaly. Abdominal ultrasonography was therefore performed, revealing hepatosplenomegaly with dilatation of the splenic vein. An abdominal computed tomography confirmed the presence of an 18.5 cm splenomegaly with dilatation of the splenic and portal vein. Upper endoscopy with biopsy showed minimal, non-atrophic, mildly active chronic, follicular antrofundic gastritis without esophageal varices. Laboratory and radiological examinations could not confirm the etiology of portal hypertension. The liver biopsy suggested hepatoportal sclerosis, compatible with IPH. The patient initially received six courses of the CTD (cyclophosphamide, thalidomide, and dexamethasone) protocol. He subsequently proceeded to an autologous stem cell transplant (ASCT), and the patient achieved a considerable improvement. POEMS syndrome could be complicated with IPH. There are only a few cases of IPH associated with POEMS syndrome in the literature. This case highlights the manifestation of portal hypertension in POEMS syndrome.
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spelling pubmed-91871352022-06-12 Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association Belabbes, Fatima Houda, Youssefi Al Bouzidi, Abderahmane Bennani, Youssef Ahnach, Maryame Cureus Pathology Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma (PCD), and at least one of the minor criteria. The most frequent liver manifestation is hepatomegaly. Idiopathic portal hypertension (IPH) has been reported rarely in POEMS syndrome. The precise etiopathogenesis of IPH is not fully elucidated. We report a 46-year-old male patient presenting with POEMS syndrome. He presented postprandial vomiting and left tinnitus. Loss of appetite, leanness, and excessive sweat were concomitant symptoms. Abdominal examination revealed splenomegaly and an absence of hepatomegaly. Abdominal ultrasonography was therefore performed, revealing hepatosplenomegaly with dilatation of the splenic vein. An abdominal computed tomography confirmed the presence of an 18.5 cm splenomegaly with dilatation of the splenic and portal vein. Upper endoscopy with biopsy showed minimal, non-atrophic, mildly active chronic, follicular antrofundic gastritis without esophageal varices. Laboratory and radiological examinations could not confirm the etiology of portal hypertension. The liver biopsy suggested hepatoportal sclerosis, compatible with IPH. The patient initially received six courses of the CTD (cyclophosphamide, thalidomide, and dexamethasone) protocol. He subsequently proceeded to an autologous stem cell transplant (ASCT), and the patient achieved a considerable improvement. POEMS syndrome could be complicated with IPH. There are only a few cases of IPH associated with POEMS syndrome in the literature. This case highlights the manifestation of portal hypertension in POEMS syndrome. Cureus 2022-05-11 /pmc/articles/PMC9187135/ /pubmed/35698702 http://dx.doi.org/10.7759/cureus.24923 Text en Copyright © 2022, Belabbes et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Pathology
Belabbes, Fatima
Houda, Youssefi
Al Bouzidi, Abderahmane
Bennani, Youssef
Ahnach, Maryame
Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association
title Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association
title_full Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association
title_fullStr Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association
title_full_unstemmed Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association
title_short Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association
title_sort polyneuropathy, organomegaly, endocrinopathy, m-protein, and skin changes (poems) syndrome and idiopathic portal hypertension: a rare association
topic Pathology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9187135/
https://www.ncbi.nlm.nih.gov/pubmed/35698702
http://dx.doi.org/10.7759/cureus.24923
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