PERINATOLOGIYA I PEDIATRIYA.2014.1(57):33-37; doi 10.15574/PP.2014.57.33 
 

Severe congenital neutropenia in children

Volokha  A. P.

P.L. Shupik National Medical Academy of Postgraduate Education, Kiev, Ukraine

Severe congenital neutropenia is a rare primary immunodeficiency with congenital defect of myeloid precursor presenting with recurrent bacterial infections in infancy. The article describes the case of severe congenital neutropenia manifested by pyogenic infections of skin, mucous membrane with complications and refractory treatment. Investigation of bone marrow revealed the arrest of neutrophil differentiation at the promyelocyte to myelocyte stage. This clinical case demonstrated difficulties of diagnostic and treatment of severe congenital neutropenia. 

Key words: severe congenital neutropenia, Kostman syndrome, children.

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