A 32-year-old man with Fanconi anemia diagnosed in childhood (13 years) who underwent transformation to acute myeloid leukemia M-5, treated with allogeneic bone marrow transplantation of an HLA sibling at age 26.
Staphiloccocus haemolyticus bacteriemia and positive antigenemia for cytomegalovirus on day 40 after transplantation, as well as iron overload requiring periodic phlebotomy.
Since then, she has been in complete remission.
There was no ethylic habit or other personal history of interest.
The current disease began with an episode of upper gastrointestinal bleeding secondary to large esophageal varices and underlying varices, on which endoscopic ligation with elastic bands was performed.
After stabilizing the patient, an abdominal procedure was performed, showing data of chronic liver disease and portal hypertension (increased caliber of the portal and quantity of ascites as well as ultrasound in moderate main flow portal).
The suprahepatic veins were normal and no lesions occupying hepatic or splenic space were found.
Physical examination revealed hepatomegaly of 3 cm and blood tests showed OGB 615 U/L, GPT 896 U/L bilirubin, AF 392 U/L esophageal varices and Gdl esophageal varices.
These laboratory abnormalities were normalized after the bleeding episode.
These findings suggested the possibility of ischemic hepatitis.
The liver disease study was completed with viral serology HAV, HBV and HCV, HIV, CMV, EBV, which were negative.
Iron, transferrin saturation, ferritin, copper, ceruloplasmin, porphyrins and alpha-1-antitrypsin were also normal.
Immunology, proteinogram and autoimmunity were normal.
An echocardiogram was normal.
Spinal cord aspirate data: findings consistent with Fanconi anemia and acute myeloid leukemia in remission with probably secondary erythroid hyperplasia.
Cytometry did not identify pathological populations.
A computed tomography scan showed new signs of chronic liver disease with homogeneous liver, as well as perivesicular varices and splenic hilium and angiolithiasis.
Vascular pathology was ruled out (portal thrombosis, Budd-Chiari syndrome, etc.).
Failing the negativity of the study of liver disease, a liver biopsy guided by ultrasound showed focal stenosis of the liver, focal ductus arteriosus, reactive ductal conformation, dilation of venous structures and endothelitis.
These findings, excluding chronic venovenous disease, chronic guest-host disease (2), and liver cirrhosis, underwent a hemodynamic study showing preo-sinus venous graft and multiple connections portal hypertension.
With the clinical and histological findings and the results of the hepatic hemodynamic study, the diagnosis of IPH was established.
