Nodular regenerative hyperplasia: evolving concepts on underdiagnosed cause of portal hypertension

Abstract

Nodular regenerative hyperplasia (NRH) is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules. NRH may lead to the development of non-cirrhotic portal hypertension. There are no published systematic population studies on NRH and our current knowledge is limited to case reports and case series. NRH may develop via autoimmune, hematological, infectious, neoplastic, or drug-related causes. The disease is usually asymptomatic, slowly or non-progressive unless complications of portal hypertension develop. Accurate diagnosis is made by histopathology, which demonstrates diffuse micronodular transformation without fibrous septa. Lack of perinuclear collagen tissue distinguishes NRH from typical regenerative nodules in the cirrhotic liver. While the initial treatment is to address the underlying disease, ultimately the therapy is directed to the management of portal hypertension. The prognosis of NRH depends on both the severity of the underlying illness and the prevention of secondary complications of portal hypertension. In this review we detail the epidemiology, pathogenesis, diagnosis, management, and prognosis of NRH.

Keywords: Comorbidities; Nodular regenerative hyperplasia; Portal hypertension.

Figure 1

Development of nodular regenerative hyperplasia and other liver adaptive reactions causing non-cirrhotic intrahepatic portal hypertension. NCIPH: Non-cirrhotic intrahepatic portal hypertension; MPD: Myeloproliferative diseases.