Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 28, 2016; 22(28): 6559-6564
Published online Jul 28, 2016. doi: 10.3748/wjg.v22.i28.6559
Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension
Cláudio Martins, Cristina Teixeira, Suzane Ribeiro, Daniel Trabulo, Cláudia Cardoso, João Mangualde, Ricardo Freire, Élia Gamito, Ana Luísa Alves, Isabelle Cremers, Cecília Alves, Anabela Neves, Ana Paula Oliveira
Cláudio Martins, Cristina Teixeira, Suzane Ribeiro, Daniel Trabulo, Cláudia Cardoso, João Mangualde, Ricardo Freire, Élia Gamito, Ana Luísa Alves, Isabelle Cremers, Ana Paula Oliveira, Department of Gastroenterology, Setúbal Hospital Center - São Bernardo Hospital, 2910 Setúbal, Portugal
Cecília Alves, Department of Pathology, Setúbal Hospital Center - São Bernardo Hospital, 2910 Setúbal, Portugal
Anabela Neves, Department of Hematology, Setúbal Hospital Center - São Bernardo Hospital, 2910 Setúbal, Portugal
Author contributions: Martins C, Cardoso C, Mangualde J, Freire R, Alves AL and Neves A designed the report; Martins C, Teixeira C, Ribeiro S and Trabulo D colected the patient's clinical data; Alves C performed histopathology examination; Martins C, Gamito E, Cremers I and Oliveira AP analyzed the data and wrote the paper.
Institutional review board statement: The study was reviewed and approved by the Setúbal Hospital Center Institutional Review Board.
Informed consent statement: The informed consent statement was waived because the patient has died.
Conflict-of-interest statement: The authors declare that they have no conflict of interests related to this work.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Cláudio Martins, Resident of Gastroenterology, Department of Gastroenterology, Setúbal Hospital Center - São Bernardo Hospital, Camilo Castelo Branco Street, Setúbal 2910, Portugal. cmartins1@campus.ul.pt
Telephone: +35-126-5549000 Fax: +35-126-5238066
Received: April 8, 2016
Peer-review started: April 8, 2016
First decision: May 27, 2016
Revised: June 7, 2016
Accepted: June 28, 2016
Article in press: June 28, 2016
Published online: July 28, 2016
Processing time: 105 Days and 8.7 Hours
Core Tip

Core tip: This clinical case describes an interesting and uncommon case of aggressive systemic mastocytosis with hepatic and gastrointestinal involvement. It illustrates not only a rare cause of non-cirrhotic portal hypertension, but also an atypical gastrointestinal involvement. The aim of this case report is to demonstrate the challenge in establishing a diagnosis of systemic mastocytosis especially when the clinical picture is atypical and without skin involvement, and alert Gastroenterologists to consider infiltrative diseases, as differential diagnosis in a clinical case of portal hypertension of unknown etiology.