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We reviewed 144 differential white blood cell counts from 23 patients with primary biliary cirrhosis (PBC, 21 females, 2 males) for occurrence of eosinophilia. Over an average observation time of 43 +/- 9 months, 9/23 (39%, 9 female) patients were found to have transient absolute (> 500/mm3, n = 5) or relative (> or = 6%, n = 4) blood eosinophilia. The maximum value of eosinophilic granulocytes observed was 1385/mm3 (19%). Other causes of eosinophilia had been ruled out. Eosinophilia was transient, disappearing without treatment. Significantly younger patients with eosinophilia also had a tendency to prognostically more favourable values for serum albumin, prothrombin time, bilirubin and galactose elimination capacity. None had edema, compared to two patients with edema in the group without eosinophilia. Two patients died during the observation period; both were from the group without eosinophilia. The average 5- and 7-year survival probability, calculated on the Mayo survival model, tended to be higher for patients with eosinophilia (92% and 87%) compared to patients without eosinophilia (79% and 72%). In patients with eosinophilia, disease stage I (5/6 vs 1/9) and epitheloid cell granuloma (4/6 vs 1/9) were significantly more frequent. Blood eosinophilia in PBC seems to be an indicator of an early disease state with florid bile duct lesions. Primary biliary cirrhosis should therefore be considered--with other hepatopathies--as a possible cause of eosinophilia of otherwise unknown origin. These observations could be of importance for future therapeutic concepts, at least for patients in early disease states.