A case of idiopathic portal hypertension that developed during the treatment for pure red cell aplasia

We herein report a case of idiopathic portal hypertension (IPH) that developed during the treatment of pure red cell aplasia (PRCA). The patient was referred to our hospital due to severe anemia at the age of 45. The findings of the bone marrow aspiration were compatible with PRCA. She was therefore treated with cy- closporine. Her serum hemoglobin level had been preserved within 8.1~12.2g/dl after the cyclosporine therapy. During the course of treatment, laboratory examinations showed no abnormalities in the levels of AST, ALT or serum albumin. Ten years after the cyclosporine therapy, when she was 55 years old, she was admitted to our hospital due to abdominal fullness. Abdominal ultrasonography and computed tomography showed an atrophic liver with massive ascites and splenomegaly, in addition to collateral veins. The histopathological findings of the liver showed that the architecture of the hepatic lobules was preserved, while the medium-sized portal veins were narrowed or occluded. Aberrant blood vessels were prominently seen. Based on these findings, a diagnosis of IPH accompanied by PRCA was made. An overlapping of IPH and PRCA is extremely rare. The present case is therefore interesting in terms of the etiology of IPH and PRCA, because autoimmune mechanisms are thought to be involved in the pathogenesis of both diseases