MYELOKATHEXIS IN A MOTHER AND INFANT - A 2ND CASE SUGGESTING DOMINANTINHERITANCE

Myelokathexis is a rare form of neutropenia that is probably congenita l, characterized by severe noncyclic neutropenia, recurrent infections , granulocytic hyperplasia of the bone marrow, and degenerative change s in mature neutrophils. The cause remains uncertain. A case of myelok athexis was reported in a father and two daughters, and based on this, myeIokathexis was given an autosomal-dominant inheritance in Mendelia n Inheritance in Man (11th edition, 1994). We present the case of a 12 -month-oId male with chronic neutropenia and the morphologic features of myelokathexis whose mother carries the same diagnosis, providing ad ditional evidence in favor of dominant transmission.