Abstract
A mother and three of her four children give evidence of lifelong persistent neutrophilia with morphologically and functionally normal granulocytes. Associated findings are hepatosplenomegaly, histiocytes of Gaucher type, thickened calvariae due to widened diploe, high leukocyte alkaline phosphatase scores, vitamin B 12 levels, heat-labile serum alkaline phosphatase activity, and a benign course. The apparent transmission from the mother to her children is consistent with an autosomal dominant mode of inheritance.
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