Abstract
A case of idiopathic portal hypertension (IPH) associated with aplastic anemia is reported. A 67-year-old Japanese woman was admitted to our hospital because of pancytopenia. She had had mild liver dysfunction for eight years. CT scan revealed huge splenomegaly and liver histology disclosed portal fibrosis with preserved lobular structures, which were consistent with IPH. Peripheral blood showed pancytopenia, and bone marrow aspiration disclosed hypoplastic marrow without myelodysplastic changes. Low uptake of the bone scintigram, high serum erythropoietin level and good response to methyl-prednisolone pulse therapy supported the diagnosis of aplastic anemia. It was speculated that immune processes were involved in the pathogenesis of IPH and aplastic anemia. Thus, coincidence of these two diseases may in part be based on common autoimmune mechanism.
