Immunotactoid hepatopathy: A novel entity with histologically proven recurrence post liver transplantation.

Immunotactoid deposition is a rare fibrillary deposition disease that is primarily seen in the kidney and is associated with paraproteinemia. Here, we report a case of hepatic immunotactoid deposition in a 67-year-old male with a history of smoldering myeloma and chronic kidney disease who underwent liver transplantation for metabolic dysfunction-related cirrhosis. Immunotactoid deposition was first identified in the explanted liver and recurred in the allograft within only 7 weeks following transplantation, presenting as ascites with normal liver function tests. The patient's posttransplant course was complicated by proteinuria and renal failure requiring dialysis. Histologic examination of both native and allograft livers demonstrated pink amorphous material occupying sinusoidal spaces that were Congo-red negative and immunoglobulin M Kappa-restricted. Electron microscopy revealed characteristic deposits of electron-dense bundles of hollow microtubules with a 40 nm diameter within the sinusoids and space of Disse, consistent with immunotactoids. Therapy of the patient's underlying plasma-cell dyscrasia utilizing a daratumumab-based regimen showed decreased serum paraproteins, resolution of ascites, and improved kidney function, no longer requiring dialysis, without inducing rejection. The patient continues to respond to treatment 10 months posttransplant.

Liver Paired Exchange: Programmatic Hopes and Fears.

The dearth of deceased liver donors has created a supply demand gap, necessitating creation of living donor liver transplantation. However, living donor liver transplantation has relied on directed donation, whereby many potential directed donors are rejected based on ABO blood group incompatibility, hepatic size incompatibility, or the need for biliary or arterial reconstruction during transplant surgery. Much like kidney paired exchange, liver paired exchange (LPE) circumvents these incompatibility issues by relying on anonymous, nondirected, or bridge donors that are better anatomical or histological matches for recipients. Although Asia has taken the lead in LPE, the process has only recently been adopted in North America, with the first successful surgery done in the United States in 2019. Our review article sheds light on the process of LPE and the success of LPE in the United States thus far and, additionally, highlights the several logistical and ethical challenges that must be considered as transplant centers adopt and scale up LPE across the United States to address the increased demand for liver allografts.

Sensitivity of ultrasound in detecting hepatocellular carcinoma in obese patients compared to explant pathology as the gold standard.

BACKGROUND/AIMS: The American Association for the Study of Liver Diseases recommends ultrasound (US) screening for hepatocellular carcinoma (HCC) among cirrhotic patients, regardless of body mass index (BMI), every 6 months. We examined US sensitivity for diagnosis of HCC in obese patients. METHODS: Liver transplant patients data with HCC in explant was used (January 2012-December 2017). All patients underwent liver US within 3 months of diagnosis of HCC. Number/size of HCC lesions were extracted from radiologic and pathologic reports. Obesity was defined as BMI ≥30 kg/m2. RESULTS: One hundred sixteen patients were included. 80% were male, with mean BMI of 31 kg/m2. The most common underlying liver disease was hepatitis C virus (62%). At the time of diagnosis, median number of HCC lesions was 2 (interquartile range [IQR], 1-3), and median size of the largest lesion was 2.5 cm (IQR, 1.75-3.9). Overall sensitivity of US study for detection of HCC was 33% (95% confidence interval [CI], 29-48%). Sensitivity was 77% (95% CI, 62-93%) in patients with BMI<30 and 21% (95% CI, 11-30%) in patients with BMI≥30 (P<0.001). Size of the largest HCC lesion (P=0.290) and number of lesions (P=0.505) were not different between groups. Computed tomography (CT) scan detected HCC in 98% of the obese patients with negative US. CONCLUSION: Sensitivity of US for detection of HCC is significantly lower among obese patients compared to overweight and normal weight patients. These patients may benefit from alternating between US and a different imaging modality.

Duct-to-duct biliary reconstruction in patients with primary sclerosing cholangitis undergoing liver transplantation.

BACKGROUND: Reconstruction of biliary drainage after liver transplantation (LTx) in patients with primary sclerosing cholangitis (PSC) has been a matter of controversy. Over recent years, the traditional method of Roux-en-Y hepaticojejunostomy (RY) has been challenged by duct-to-duct (DD) biliary reconstruction. METHODS: This study represents a retrospective review of biliary complications, patient and graft survival after LTx in PSC patients based on type of biliary reconstruction. Outcomes of DD reconstruction in this group of patients and non-PSC patients are compared. RESULTS: A total of 53 primary LTx procedures were performed for PSC between August 2005 and July 2010. Seven patients were excluded because unexpected cholangiocarcinoma was found in the explants (n=3) or because they received partial livers (n=4). Biliary reconstruction was performed as DD in 18 patients and RY in 28 patients. There were no bile leaks. Anastomotic stricture occurred in two (11%) patients in the DD group and one (4%) in the RY group. Two (7%) patients in the RY group developed non-PSC intrahepatic strictures and one had recurrence of PSC. Rates of 1- and 3-year patient and graft survival in the RY and DD groups were 96.7% and 96.7%, and 100% and 94.5%, respectively. In a group of 34 randomly selected patients transplanted for a non-PSC diagnosis with DD reconstruction during the same period, the anastomotic stricture rate was 9% and 1- and 3-year patient and graft survival rates were 97.0% and 88.5%; differences were not significant. CONCLUSIONS: Duct-to-duct biliary reconstruction at the time of LTx in selected PSC patients is both effective and safe, and shows outcomes comparable with those of RY reconstruction in these patients and those of DD reconstruction in non-PSC patients.