Abstract

Introduction: Histoplasma capsulatum is the most common endemic mycosis in the United States. Extrapulmonary hepatic histoplasmosis as a primary manifestation is extremely rare. We report a case of histoplasmosis that presented like chronic liver disease with portal hypertension, splenomegaly, esophageal varices, and thrombocytopenia in an immunocompetent adult. Case Description: A 56 year-old Caucasian man from West Virginia who works in construction presented to the hepatology clinic for work up of chronic liver disease. He was initially evaluated by his primary care doctor for complaints of diarrhea and had an EGD performed that revealed esophageal varices. On examination, he was noted to have splenomegaly but no other stigmata of chronic liver disease. A CBC was only remarkable for a platelet count of 86. A CMP was unremarkable with normal liver enzymes. INR was 1.0. Serological work up for liver disease including ceruloplasmin, remote hepatitis panel, iron studies, anti-nuclear antibody, anti-mitochondrial antibody, anti-smooth muscle antibody, and alpha 1 anti-trypsin were normal. A RUQ ultrasound with elastography showed a normal, homogenous liver, splenomegaly and a liver stiffness of F2-F3 indicating moderate fibrosis. EGD was repeated which showed large (>5mm) esophageal varices that were banded. It also revealed moderate portal hypertensive gastropathy. Transjugular liver biopsy was performed that revealed bridging fibrosis with minimal steatosis, no obvious granulomas but loose clusters of histiocytes, and a gomori methenamine silver stain was performed looking for histoplasmosis that highlighted small yeast with budding typical for Histoplasma capsulatum. Histoplasma antibody in the blood was positive. The patient was seen by infectious disease and he was started on oral itraconazole. Discussion: Hepatic histoplasmosis can present as granulomatous hepatitis, as stigmata of portal hypertension, including ascites or varices, or with elevated liver enzymes. The most common hepatic finding on liver biopsy is portal lymphohistiocytic inflammation; discrete hepatic granulomas are seen in less than 20% of involved livers. There are only a few case reports of disseminated histoplasmosis presenting as primary hepatic histoplasmosis. A high clinical suspicion is warranted in patients who present with stigmata of portal hypertension and chronic liver disease with no evidence of cirrhosis, especially if the person lives in or has visited an endemic area.2252 Figure 1. The normal lobular architecture is distorted by bridging fibrosis. The hepatocytes show minimal steatosis. Although well-developed granulomas are not identified, there are loose clusters of histiocytes.

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