Idiopathic portal hypertension associated with polyclonal hyperimmunoglobulinemia. A case report and review of the literature.

We report a case of idiopathic portal hypertension associated with prominent polyclonal hyperimmunoglobulinemia and plasmacytosis in the bone marrow and the spleen. Microscopic examination of the liver showed chronic inflammation in the portal area and abnormal vasculature adjacent to the portal tracts. In the spleen, polyclonal plasma cell proliferation was demonstrated histochemically in addition to the presence of remarkable sinus hyperplasia and periarterial fibrosis. In the present case, a chronic inflammatory state such as connective tissue disease was strongly suspected to exist in the liver, since pancytopenia and hyperimmunoglobulinemia persisted even after splenectomy. It is suggested that the pathogenesis in the present case may have been due to this chronic inflammatory state.