Mj. Christ et Ca. Dillon, MYELOKATHEXIS IN A MOTHER AND INFANT - A 2ND CASE SUGGESTING DOMINANTINHERITANCE, Military medicine, 162(12), 1997, pp. 827-828
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.