Case Report
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World J Gastroenterol. May 28, 2011; 17(20): 2580-2584
Published online May 28, 2011. doi: 10.3748/wjg.v17.i20.2580
Non-cirrhotic portal hypertension with large regenerative nodules: A diagnostic challenge
Umberto Vespasiani Gentilucci, Paolo Gallo, Giuseppe Perrone, Riccardo Del Vescovo, Giovanni Galati, Sandro Spataro, Chiara Mazzarelli, Adriano Pellicelli, Antonella Afeltra, Antonio Picardi
Umberto Vespasiani Gentilucci, Paolo Gallo, Giovanni Galati, Sandro Spataro, Chiara Mazzarelli, Antonella Afeltra, Antonio Picardi, Clinical Medicine and Hepatology Unit, University Campus Bio-Medico of Rome, 00128 Rome, Italy
Giuseppe Perrone, Clinical Pathology, University Campus Bio-Medico of Rome, 00128 Rome, Italy
Riccardo Del Vescovo, Department of Radiology, University Campus Bio-Medico of Rome, 00128 Rome, Italy
Adriano Pellicelli, Hepatology Unit, San Camillo-Forlanini Hospital, 00152 Rome, Italy
Author contributions: All authors contributed equally to this work; Vespasiani Gentilucci U and Gallo P wrote the paper.
Correspondence to: Umberto Vespasiani Gentilucci, MD, Clinical Medicine and Hepatology Unit, University Campus Bio-Medico of Rome, Via Alvaro del Portillo, 200, 00128 Rome, Italy. u.vespasiani@unicampus.it
Telephone: +39-6-225411  Fax: +39-6-22541456
Received: November 27, 2010
Revised: February 15, 2011
Accepted: February 22, 2011
Published online: May 28, 2011
Abstract

Non-cirrhotic portal hypertension is a poorly understood condition characterized by portal hypertension in the absence of conventional hepatic cirrhosis and described in association with blood coagulation disorders, myeloproliferative and immunological diseases and with exposure to toxic drugs. Very recently, precise classification criteria have been proposed in order to define four distinct subcategories. The present case highlights how the clinical presentation, the confounding results from imaging studies, and the difficulties in the histological evaluation often render cases of non-cirrhotic portal hypertension a real diagnostic challenge. It also underscores the classification problems which can be faced once this diagnosis is performed. Indeed, the different subcategories proposed result from the prevalent subtypes in a spectrum of hepatic regenerative responses to a variety of injuries determining microcirculatory disturbances. More flexibility in classification should derive from this etiopathogenic background.

Keywords: Non-cirrhotic portal hypertension, Large regenerative nodules, Nodular regenerative hyperplasia