BOALA KOSTMANN LA COPIL
Kostmanns disease (KD) is a severe
congenital neutropenia, a rare autosomal recessive disorder of neutrophil number. Complete
blood count with differential reveals an ANC less than 500/mm3, as seen in classic cases,
monocytosis and eosinophilia. Bone marrow aspiration or biopsy reveals an arrest of neutrophil
precursor maturation at the promielocyte or myelocyte level. KD results in an increased
susceptibility to frequent bacterial infections. In the absence of medical intervention with
granulocyte colony-stimulating factor (G-CSF), bone marrow transplantation, the mortality rate
is 70% within the first year of life. We present three cases to illustrate this rare entity, the
difficulty of diagnosis, but also to underline the importance of correct treatment.
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