Abstract
The patient with common variable immunodeficiency and with new onset progressive splenomegaly was hospitalized for diagnostics. Infections, hematological diseases, lysosomal storage diseases were excluded.
There were signs of portal hypertension, without liver leasion, checked by computer tomography and gastroscopy. Histological examination of the liver excluded hepatic cirrhosis.
Based on the presence of nodules characteristic of nodular regenerative hyperplasia, the case was concluded as idiopathic non-cirrhotic portal hypertension. INCPH is an unusual liver disease leading to a nodulated appearance of the liver with portal hypertension, while this combination can easily be misdiagnosed as liver cirrhosis.
It is associated with some immunopathology and procoagulant situations. Patophysiologicaly there are thrombosis of small intrahepatic branches of the portal vein.
Diagnosis is done by histology, where nodular regenerative hyperplasia and cirrhosis with incomplete sepsis, as well as portal vein stenosis, portal vein herniation, hypervascularized portal field and abnormal periportal vessels are characteristic leasion. Some patients can be treated with anticoagulation therapy.