Liver transplantation for the treatment of nodular regenerative hyperplasia

Manzia, Tommaso Maria; Gravante, Gianpiero; Paolo, D. Di; Orlando, Giuseppe; Toti, Luca; Bellini, Maria Irene; Ciano, Paolo; Angélico, M.; Tisone, Giuseppe

doi:10.1016/j.dld.2011.04.004

Cited by 23 publications

(29 citation statements)

References 71 publications

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“…Similar to OPV, nodular regenerative hyperplasia (NRH) often presents as portal hypertension in the absence of cirrhosis. The most frequent clinical presentation is gastroesophageal bleeding (65.3%) followed by ascites (61.5%), hepatic encephalopathy (30.7%) and liver failure (11.5%) 13 . Rarely, some patients may need liver transplantation 13 .…”

Section: Introductionmentioning

confidence: 99%

Aberrant von Willebrand factor expression of sinusoidal endothelial cells and quiescence of hepatic stellate cells in nodular regenerative hyperplasia and obliterative portal venopathy

et al. 2020

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2020) Histopathology 76, 959-967. https://doi.org/10. 1111/his.14083 Aberrant von Willebrand factor expression of sinusoidal endothelial cells and quiescence of hepatic stellate cells in nodular regenerative hyperplasia and obliterative portal venopathy Aims: Nodular regenerative hyperplasia (NRH) and obliterative portal venopathy (OPV), entities that comprise idiopathic non-cirrhotic portal hypertension (INCPH), are under-recognised diseases of uncertain aetiology and the diagnosis can be easily missed on liver biopsy. The expression of CD34 and von Willebrand factor (vWF) in liver sinusoidal endothelial cells (LSEC) and alpha-smooth muscle actin (ASMA) in hepatic stellate cells (HSCs) is unknown in NRH and OPV. We sought to investigate the pathogenesis and potential immunomarkers that might aid in making the diagnosis of NRH and OPV. Methods and results: Immunohistochemical (IHC) staining for CD34, vWF and ASMA was performed in clinically and histologically well-characterised NRH (n = 15) and OPV (n = 47) liver specimens. Among the 47 OPV cases, 37 (78.7%) had concurrent fea-tures of NRH. CD34 positive staining was mainly confined to small vessels in the portal tracts and LSECs in periportal areas, a finding similar to that in non-NRH/OPV livers. However, expression of vWF in LSECs was positive in the compressed sinusoids of NRH and in a patchy or geographic pattern, particularly prominent in the perivenular areas and dilated sinusoids of OPV cases. HSCs were negative for ASMA in all NRH and OPV cases. Conclusion: Our findings indicate that NRH may be a subtle but common concurrent morphological feature in OPV. The aberrant expression of vWF in LSECs suggests that endothelial injury may play a role in the pathogenesis, which may thus aid in the recognition and diagnosis of NRH and OPV, particularly when confronted with otherwise apparent normal liver histology on needle biopsy.

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Section: Introductionmentioning

confidence: 99%

Aberrant von Willebrand factor expression of sinusoidal endothelial cells and quiescence of hepatic stellate cells in nodular regenerative hyperplasia and obliterative portal venopathy

et al. 2020

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“…The term NRH was first introduced in 1959 by Steiner . It is defined by the presence of hepatocellular nodules, usually smaller than 3 mm; however, histologically, this nodular appearance throughout the liver is not due to increased fibrosis or cirrhosis at all. The diffuse nodularity rather comes from small and atrophic hepatocytes often located at zone 3.…”

Section: Introductionmentioning

confidence: 99%

“…8,11,14 A spectrum of systemic disease is associated with NRH as a manifestation of chronic liver damage in settings of autoimmune diseases, and myeloproliferative and lymphoproliferative disorders. 8,9,12,[15][16][17][18][19][20][21][22] The development of DnNRH has been previously described in the adult population and has been correlated with the use of azathioprine and the subsequent development of portal hypertension. 12,[22][23][24][25] Devarbhavi et al reported DnRNH in 14 patients in a cohort of 1191 liver transplant patients, giving an incidence of approximately 1%; one patient was a child.…”

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Clinicopathologic characteristics of de novo nodular regenerative hyperplasia in pediatric liver transplant

González

Ritter

et al. 2019

Pediatric Transplantation

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Liver NRH is seen in all patients age; however, more frequently in those over the age of 60 years and associated with multiple systemic diseases. In liver allograft recipients, the development of DnNRH has been linked with the use of azathioprine or vascular abnormalities. We present the clinicopathologic characteristics of 17 pediatric patients who underwent liver transplantation and subsequently developed DnNRH. The patients were divided into early and late onset depending if DnNRH was diagnosed within or beyond 4 years after transplant. Eight patients (47%) presented as early onset, of which two had normal ultrasound at time of diagnosis. One patient (12.5%) with early onset lost the graft secondary to DnNRH. Nine patients (53%) presented as late onset, of which two (22%) had normal ultrasound at time of diagnosis. Two patients (25%) of the late onset lost their graft secondary to chronic rejection and DnNRH. Two patients (12%) died secondary to cytomegalovirus pneumonitis and pancolitis. Furthermore, both groups presented with symptoms differing from the adult population in prior studies and were not associated with the use of azathioprine or vascular abnormalities. Interestingly, episodes of acute cellular rejection were more common in the early‐onset group compared to the late‐onset group. In conclusion, DnNRH in the pediatric age group has a different clinical presentation, possibly reflecting a different pathogenesis compared to the adult population.

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“…One meta-analysis identified 17 studies in which liver transplantation was considered as a treatment for NRH with severe portal hypertension refractory to conventional therapies. 16 Twenty-six patients received liver transplantation. The most frequent indications were esophageal variceal bleeding (17/26; 65.3%), ascites (16/26; 61.5%), encephalopathy (8/26; 30.7%), and spontaneous bacterial peritonitis (1/26; 3.8%).…”

Section: Discussionmentioning

confidence: 99%

Nodular Regenerative Hyperplasia

Lee

Izzy

Akki

et al. 2017

Journal of Investigative Medicine High Impact Case Reports

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Introduction: Nodular regenerative hyperplasia (NRH) is a known etiology of noncirrhotic portal hypertension. Cases of biopsy-proven NRH in human immunodeficiency virus (HIV)–positive patients have been described. While these patients often have normal synthetic liver function, several reports described disease progression to liver failure. Case: We here present a 26-year-old woman with history of congenital HIV on antiretroviral therapy complicated by Pneumocystis carinii pneumonia at age 14. CD4 counts have been >300 with undetectable viral load. She was referred to our Hepatology service for evaluation of splenomegaly, elevated liver tests, and thrombocytopenia. On initial presentation, she reported easy bruising and gingival bleeding, and abdominal imaging showed evidence of portal hypertension without associated cirrhosis. Upper endoscopy was significant for large esophageal varices without bleeding stigmata. Liver biopsy showed minimal fibrosis around the portal areas without significant inflammation. The lobules showed focal zones of thin hepatocyte plates on reticulin stain with adjacent areas showing mild regenerative changes. The diagnosis of NRH was made and patient was placed on propranolol for variceal bleeding prophylaxis. Two years later, the patient presented with bleeding gastric varices warranting transjugular intrahepatic portosystemic shunt. Postprocedure course was complicated by mild encephalopathy. Subsequent magnetic resonance imaging showed a 1.7 × 1.3 cm lesion suggestive of hepatocellular carcinoma (HCC). The patient was deemed to be a candidate for liver transplantation, and she is now delisted due to ongoing pregnancy. Conclusion: This report describes the first case of HCC in an HIV patient with NRH. The possible association of NRH with HCC warrants further investigation.

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Liver transplantation for the treatment of nodular regenerative hyperplasia

Cited by 23 publications

References 71 publications

Aberrant von Willebrand factor expression of sinusoidal endothelial cells and quiescence of hepatic stellate cells in nodular regenerative hyperplasia and obliterative portal venopathy

Aberrant von Willebrand factor expression of sinusoidal endothelial cells and quiescence of hepatic stellate cells in nodular regenerative hyperplasia and obliterative portal venopathy

Clinicopathologic characteristics of de novo nodular regenerative hyperplasia in pediatric liver transplant

Nodular Regenerative Hyperplasia

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