Can spherocytosis cause an itch?
J. TOOP, MD, of Martintown, ON, writes: "My 87-year-old patient has congenital spherocytosis. His bilirubin is quite elevated and he's itchy. Ursodiol and doxepin didn't help. Could you please elaborate on this disease?"
It's quite unusual to see an elderly patient with congenital spherocytosis and jaundice who hasn't had a splenectomy. If the individual did have his spleen removed, he shouldn't have high bilirubin due to hemolysis as part of this disease. In any case, whether the condition is the result of spherocytosis or some other hemolytic anemia, it's not associated with itching. Pruritus linked to a high serum bilirubin is due to obstructive jaundice that, while increasing the direct bilirubin in the blood, causes the retention of bile salts.
Your patient with hereditary spherocytosis and hyperbilirubinemia with itching must have an obstructive jaundice. If he still has his spleen, this may be due to the presence of a bilirubin stone in the common bile duct or to an unrelated condition, e.g. carcinoma of the head of the pancreas, lymphoma, etc. He should be properly investigated and referred to a surgeon or gastroenterologist, depending on the cause of the obstruction. MS