Year 2017 / Volume 109 / Number 5
Letter to the Editor
POEMS syndrome and idiopathic portal hypertension: a possible association

393

DOI: 10.17235/reed.2017.4623/2016

Sara Campos, Cláudia Agostinho, Maria Augusta Cipriano,

Abstract
A 48-year old female patient was admitted to the emergency department with upper gastrointestinal bleeding. Endoscopy showed large esophageal varices that were treated with band ligation. She had been treated with cyclophosphamide, melphalan, lenalidomide and corticosteroids for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy or edema, M protein, skin changes). She had no other risk factors for chronic liver disease. Laboratory and radiological examinations could not confirm the etiology of portal hypertension. The liver biopsy suggested hepatoportal sclerosis, compatible with idiopathic portal hypertension (IPH). Under a band ligation program, with beta-blocker, diuretics and prophylactic anticoagulation, the patient remains stable. In POEMS syndrome, a rare multi-systemic disease, the most frequent liver manifestation is hepatomegaly(1). To the best of authors' knowledge, IPH has been reported only 3 times in POEMS syndrome(2,3,4) and this is the second case reporting the occurrence of histological findings compatible with IPH. As the precise etiopathogenesis of IPH is not fully elucidated and as it is an extremely uncommon manifestation in POEMS, it is not yet certain whether IPH is part of the syndrome or whether it develops independently. While some investigators believe that there is a congenital vascular anomaly involving the changes in the portal tract, the majority support an acquired vascular defect hypothesis(5). The acquired IPH hypothesis emphasizes a possible role of various pathogenic determinants: infections, prothrombotic states, prolonged exposure to several medications and toxins, immunological basis and progressive fibrosis of the portal veins (5). In this hypothesis, a hepatic circulation defect secondary to POEMS syndrome, which includes pro-inflammatory (VEGF, interleukin (IL)-6, IL-1ß, tumor necrosis factor-α, tumor growth factor-ß) and pro-thrombotic factors (aberrations in the thrombin-antithrombin complex)(1) which have been documented in the IPH and POEMS syndrome, may contribute to a possible association between these entities. Although the association between these two entities is yet to be confirmed, the authors present this case in order to contribute to its elucidation.
Share Button
New comment
Comments
No comments for this article
References
1- Dispenzieri A. POEMS syndrome: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol. 2014; 89:214–23.
2- Teramoto J, Itoh R, Inagaki T, et al. A case of polyneuropathy with monoclonal gammopathy, hepatosplenomegaly, ascites, skin pigmentation, and swelling of salivary glands. Neurol Med. 1977; 7:500–3.
3- Stepani P, Courouble Y, Postel P, et al. [Portal hypertension and neutrocytic ascites in POEMS syndrome]. Gastroentérologie Clin Biol. 1998; 22:1095–7.
4- Inoue R, Nakazawa A, Tsukada N, et al. POEMS syndrome with idiopathic portal hypertension: autopsy case and review of the literature. Pathol Int. 2010; 60:316–20.
5. Kondo F. Benign nodular hepatocellular lesions caused by abnormal hepatic circulation: etiological analysis and introduction of a new concept. J Gastroenterol Hepatol. 2001; 16:1319–28.
Related articles

Editorial

Treatment of portal vein thrombosis in cirrhosis patients

DOI: 10.17235/reed.2023.9800/2023

Letter

Tolvaptan in portal hypertension: real life experience

DOI: 10.17235/reed.2022.9145/2022

Letter

Hemoperitoneum due to rupture of intra-abdominal varices

DOI: 10.17235/reed.2022.8937/2022

Digestive Diseases Image

Traumatic high flow arterioportal fistula. Correction by a covered stent

DOI: 10.17235/reed.2022.8724/2022

Letter

Stomal varices: an unusual cause of bleeding in liver cirrhosis

DOI: 10.17235/reed.2020.6470/2019

Letter to the Editor

Idiopathic portal hypertension with regard to thiopurine treatment

DOI: 10.17235/reed.2018.5256/2017

Citation tools
Campos S, Agostinho C, Cipriano M. POEMS syndrome and idiopathic portal hypertension: a possible association. 4623/2016


Download to a citation manager

Download the citation for this article by clicking on one of the following citation managers:

Metrics
This article has received 394 visits.
This article has been downloaded 251 times.

Statistics from Dimensions


Statistics from Plum Analytics

Publication history

Received: 17/09/2016

Accepted: 25/09/2016

Online First: 01/03/2017

Published: 03/05/2017

Article Online First time: 165 days

Article editing time: 228 days


Share
This article hasn't been rated yet.
Reader rating:
Valora este artículo:




Asociación Española de Ecografía Digestiva Sociedad Española de Endoscopia Digestiva Sociedad Española de Patología Digestiva
The Spanish Journal of Gastroenterology is the official organ of the Sociedad Española de Patología Digestiva, the Sociedad Española de Endoscopia Digestiva and the Asociación Española de Ecografía Digestiva
Cookie policy Privacy Policy Legal Notice © Copyright 2023 y Creative Commons. The Spanish Journal of Gastroenterology