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Sir.—I read with interest the article "Primary Sclerosing Cholangitis Associated With Immunodeficiency" by Naveh and colleagues (Journal 1983; 137:114-117). Their young patient with this rare syndrome, in addition to dysgammaglobulinemia, had a high reticulocyte count (8.8%) at 1 year of age despite a normal hemoglobin level (12.7 g/dL). This might indicate that he also had compensated hemolytic anemia with eosinophilia. Despite hemolytic anemia and primary biliary duct involvement, his serum bilirubin level was slightly elevated, but the unconjugated fraction was not elevated. From the early appearance of his disease, I wonder how much his hemolytic anemia was responsible for causing early bile plug syndrome.
OZSOYLU S. Hemolytic Disease. Am J Dis Child. 1983;137(11):1131. doi:10.1001/archpedi.1983.02140370087036