title: Abnormalities of the spleen and liver in patients with hemophilia.
abstract: Previous studies have demonstrated functional and histologic abnormalities of the liver, and, more recently, splenomegaly in patients with hemophilia. Since these observations usually were derived from hemophiliacs who had received intensive replacement therapy, the question was posed as to whether the frequency of splenic and hepatic abnormalities was secondary to the amount of therapy utilized. In this study, a variety of tests were employed to evaluate spleen and liver size and function to determine if abnormalities in these organs correlated with the intensity of the transfusion program. The study group was comprised of 25 hemophiliacs (mean factor replacement-18,361 U/year; median factor replacement-12,920 U/year). Over 70% of our patients had elevations of aspartate and alanine aminotransferase. Immunoglobulin and complement levels were normal in most subjects. Ninety-six percent had evidence of exposure to hepatitis B virus. Liver-spleen imaging suggested significant hepatic abnormalities in most of the patients as evidenced by inhomogeneity of tracer uptake in the liver in 33% and relatively increased colloid uptake in the spleen in 90%. Splenomegaly (palpable spleen or enlargement on liver-spleen imaging) was detected in 40% of our patients, and tended to occur in the more frequently transfused patients. These findings indicate that significant abnormalities of the spleen and liver can occur in hemophiliacs who have received moderate amounts of replacement therapy and that liver-spleen imaging may be a useful method for monitoring the development of hepatic and splenic abnormalities.
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D006467:Hemophilia A / Q000150:complications* / Q000276:immunology / Q000628:therapy; D006505:Hepatitis / Q000209:etiology; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D008099:Liver / Q000000981:diagnostic imaging; D008107:Liver Diseases / Q000209:etiology*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D011877:Radionuclide Imaging; D013154:Spleen / Q000000981:diagnostic imaging; D013158:Splenic Diseases / Q000209:etiology*; D013163:Splenomegaly / Q000209:etiology
chemical_list: D007136:Immunoglobulins