Histopathological Study of Non-Cirrhotic Portal Fibrosis (NCPF) With Special Emphasis on Advanced Fibrosis

Abstract

Background: Non-cirrhotic portal hypertension includes primarily the cases of non-cirrhotic portal fibrosis (NCPF) and extra-hepatic portal vein obstruction, both showing no significant parenchymal disease. NCPF is a disease of uncertain etiology characterized by periportal fibrosis with small and medium sized portal vein branches involvement. Most study done in the past are clinical, hence we decided to analyse its histo-pathological features with clinical correlation. Methods: This is four and half year's retrospective study of liver biopsies of clinically diagnosed patients of NCPF. Fifteen patients of EHPVO served as controls. Biopsies were stained with Haematoxylin and eosin, reticulin and Masson's trichrome. Result: Total 978 benign specimens (excluding space-occupying lesions) received, of which 45 diagnosed as NCPF (4.6%). Most patients (68.88%) were between 20-40 years (M:F ratio 1:1.5), commonest symptoms long-standing splenomegaly (95%), anemia (78%) and variceal bleeds (42%) with duration varying from 3 months to 11 years. Commonest histopathological findings were portal tract fibrosis (77.78%), phlebosclerosis (68.89%), portal tract remnants (24.44%), more frequently seen in NCPF than EHPVO, septal fibrosis was seen in five. Follow up available in 91.11% cases showed no mortality or liver failure, but significant morbidity due to repeated episodes of variceal bleeds and hypersplenism. Conclusion: NCPF is still common in India. Distinctive features on histology are portal tract fibrosis, portal tract remnants, with phlebosclerosis which is being statistically significant when comparing with EHPVO. NCPF can show advanced fibrosis, can also present with features of decompensation mimicking cirrhosis, however its course remains stable with good long-term survival.

Variations in Hepatic Vein Pressure Gradient and Liver Stiffness in the Histological Categories of Non-cirrhotic Portal Hypertension

“…Previously, a study of NCPH from our group showed the median hepatic venous pressure gradient (HVPG) and liver stiffness (LS, Fibroscan) values were 8 mm of Hg (range: 5-20 mm of Hg) and 9.2 kPa (range: 4.4-26.3 kPa), respectively [7]. This study indirectly indicated that a subset of patients with NCPH can have high HVPG and TE.…”

“…The categories of NCPH based on histological characteristics [5][6][7][8] that were used for the categorization into appropriate entities are tabulated in Table 1. Cases were classi ed a priori into six categories: obliterative portal venopathy (OPV), obliterative portal venopathy with brosis (OPV-F), incomplete septal cirrhosis (ISC), nodular regenerative hyperplasia (NRH), megasinusoids with brosis (MSF), [Fig.…”

“…However, since the NCPH is a broad term with many histological features and subcategories. [5][6][7][8] At times, it can have elevated LS and HVPG and be confused with liver cirrhosis. [8,9] In this study, we aimed to categorise those entities of NCPH that may show variations in LS and HVPG and have higher LS and HVPG values for prognostic relevance.…”