#6841 IS NEPHRECTOMY ALWAYS USEFUL IN ADPKD? A CASE REPORT OF HEPATIC FIBROSIS WITH SPLENOMEGALY AND PANCYTOPENIA AS LATE CONSEQUENCE
Authors: Kola Kristiana , De Rosa Liliana , Catania Martina , Vespa Marta , Bucci Romina , Joli Giancarlo , Paolisi Michele , Bianca Pierpaolo , Vezzoli Giuseppe , Manunta Paolo , Alibrandi Maria Teresa Sciarrone 
Published: June 2023

Abstract

Background and Aims
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease. Its main feature is the progressive enlargement of both kidneys with progressive loss of kidney function. ADPKD is the 4th leading cause of dialysis in the world. The disease has systemic involvement. Liver cysts are the most common extrarenal manifestations of ADPKD and are often incidental findings. However the liver can reach considerable size but its function is always preserved. Cysts lead to structural changes of the biliary tree and determine discomfort related to mass effect when significant liver enlargement occurs. Few cases of Budd Chiari syndrome (BCS) or of hepatic fibrosis have been reported in ADPKD patients after nephrectomy. Probably the removal of the kidneys can lead to obstruction of inferior vena cava by liver cysts.

Method
We report a case of hepatic fibrosis with splenomegaly and severe pancytopenia as a tardive complication after bilateral nephrectomy.

Case report
A 47 years old man was admitted c/o Division of Nephrology of San Raffaele Hospital in August 2018 because of a high-grade fever, general malaise, nausea and abdominal pain. The patient had a previous diagnosis of ADPKD. Genetic test indicative of PKD1 truncating mutation. He was chronically attended in the outpatient clinic of the same Division. On admission in hospital his renal function was severely impaired (crea 10 mg/dl; urea 213 mg/dl). No abnormalities at the blood count. Reactive C protein was elevated (156 mg/l nv 0-6). Urine culture was found negative. Abdominal NMR showed an important volumetric increase (compared with a NMR performed in 2014) of both kidneys with diameters of about 30 cm, several cysts with hemorrhagic and infective features and numerical and dimensional increase of the hepatic cysts; spleen was reported with normal diameters. Multiple antibiotic treatments were performed unfortunately without benefit. Therefore considering the state of end stage renal disease, the persistent infection and the severe symptoms, in September 2018 the patient underwent bilateral nephrectomy and hemodialysis was started. During the following years imaging studies aimed at the preparation for transplantation were performed. In November 2020 an abdominal NMR showed for the first time splenomegaly with a bipolar diameter of 14 cm. A NMR follow up was started. In January 2021 a further increase was reported as spleen diameter resulted of 14.5 cm and a volumetric increase of hepatic cysts was shown. In September 2022 spleen reached to a diameter of 17 cm. Contextually in November 2020 patient started to present pancytopenia that had progressively been worsening with many concomitant infective and bleeding episodes. All hematologic causes were excluded. Just to explain the genesis of the splenomegaly in September 2022 a fibroscan was performed and a pathological liverstiffness was found. All hepatitis markers were negative and immunologic ones as well. Splenectomy was assumed, but patient clinical conditions so far do not allow it. Transplantation is currently contraindicated

Conclusion
These evidences underline the necessity of scrupolous evaluation of polycystic kidneys and their anatomic relationship with the liver before nephrectomy. Nephrectomy is frequently considered in the preparation for renal transplantation. In light of these possible complications, maybe removal of kidneys should be considered carefully in ADPKD patients with large involvement of the liver by cysts.