Nodular Regenerative Hyperplasia After Liver Transplant; It’s All in the Presentation

Abstract

There is a paucity of data on nodular regenerative hyperplasia after liver transplant. We aim to define the clinical disease trajectory and identify predictors of outcome for this rare diagnosis. This is a retrospective review of postulated risk factors and outcome in patients with nodular regenerative hyperplasia. Patients were classified as having a late presentation if nodular regenerative hyperplasia was diagnosed > 48 months from transplant, and symptomatic if portal hypertensive symptoms were present. Forty-nine of 3,711 (1.3%) adult recipients developed nodular regenerative hyperplasia, and mortality was 32.7% with an average follow up of 84.6 months. The MELD-Na 6 months after diagnosis did not change significantly. Patients with symptomatic portal hypertension at the time of diagnosis had a significantly higher risk of mortality (51.8%) compared to patients with liver test abnormalities alone (10.5%). 44.9% of patients had no previously postulated risk factor. Anastomotic vascular complications do not appear to be the etiology in most patients. The results suggest the vast majority of patients presenting with liver test abnormalities alone have stable disease and excellent long term survival, in contrast to the 56.3% mortality seen in patients that present more than 48 months after LT with symptomatic portal hypertension at diagnosis.

Nodular regenerative hyperplasia and liver transplantation: a systematic review

“…Complications of NRH development after liver transplantation ranged in severity from changes in liver function to the development of portal hypertension. There are reports of intervening on vascular abnormalities, with variable follow-up as to the persistence or resolution of NRH ( 33 , 38 , 39 ). Some patients required endovascular or surgical portocaval shunts for the treatment of portal hypertension ( 40 ).…”

Nodular Regenerative Hyperplasia Is Not a Rare Condition After Liver Transplantation: Incidence, Predictive Factors, and Impact on Survival

“…3,33,52 The effects of blood flow on vessels, and particularly on endothelial cells, are mainly due to wall shear stress, which can involve a multitude of physical and molecular mechanisms. 34,35,[53][54][55] However, it is difficult to know if portal thrombosis is the consequence or the cause of NRH.…”

Evidence for Alloimmune Sinusoidal Injury in De Novo Nodular Regenerative Hyperplasia After Liver Transplantation

“…Most cases are asymptomatic, diagnosed on protocol liver biopsies (LB); however, some cases require retransplantation due to portal hypertension. Of the etiologies for NRH in native liver, azathioprine and vascular issues are higher up on the list of differential causes, however the number of unexplained cases remains high [ 11 ]. Porto-sinusoidal vascular disease (PSVD), which produces NRH is reported as an uncommon cause of recurrent disease after LT [ 14 ].…”

“…HLA DSA testing is haphazard, hepatologist-dependent, and non-HLA DSA is even less frequently tested. In the most recent series [ 11 ], based on a chart review without looking for histological clues to the potential etiology, no unifying risk factors were found, but the data pointed towards an immune-mediated process in the development of NRH.…”

“…The development of NRH following liver transplantation (LT) is not well documented, with only a few case reports [5][6][7][8] and three retrospective series in adult recipients [9][10][11]. NRH is seen with increasing frequency and with increased graft longevity [12,13].…”