Idiopathic Portal Hypertension Related Refractory Ascites (IPHRA) after Allogeneic Stem Cell Transplant

Abstract

Background At our center, we observed a series of patients who developed transudative refractory ascites secondary to non-cirrhotic non-veno-occlusive disease (NC-nVOD) portal hypertension after allogeneic transplant (allo-SCT). The natural history, pathogenesis and etiology of this phenomenon is not well characterized nor well studied in the literature. Objectives To study and analyze the clinical characteristics and outcome of myeloid and lymphoid malignancy patients who developed IPHRA post allo-HCT. Methods In this retrospective study, we analyzed age>18 myeloid and lymphoid malignancy patients who developed IPHRA post allo-HCT. IPHRA was defined as NC-nVOD intrahepatic portal hypertension (serum ascites albumin gradient ≥ 1.1 or hepatic venous pressure gradient (HVPG) >5) and related refractory ascites in the absence of pre and post-hepatic etiologies of portal hypertension. Results We identified 24 patients who developed IPHRA post allo-HCT from our database. Median age at the time of allo-HCT was 52 yrs (range, 23-67). 23/24 did not have elevated LFT (Transaminases> 2.5 ULN/Bilirubin>1.5 ULN) before allo-HCT. Median time to development of IPHRA after allo-HCT was 95 days (range, 21-576 days). A median number of paracentesis that these patients had was 2 (range, 1-11); 5 had an intraperitoneal catheter placed, and 2 had TIPS to decrease the portal pressure. Portal pressures were measured in 15 patients, 9 had moderate portal hypertension (HVPG 5-10), and 6 had severe portal hypertension (HVPG >10). Liver biopsy was done in 20 patients, and for 3 patients we had autopsy results available. None of the patients met either the histological or clinical criteria of VOD or cirrhosis. 19 patients (79 %) died, and the median survival after development of IPHRA was 5.2 months (range, 0.7-49). The most common cause of death were complications of ascites/liver failure (n=7, 37%), and infectious causes (n=7, 37%). Conclusion IPHRA is an unrecognized fatal entity post-allo-HCT and has a high mortality rate. Further investigation is necessary to establish prognostic criteria for patients at risk of this serious complication.