Document Type
Article (Restricted)
Publication Date
1-1-2005
Abstract
Kostmann's syndrome is a rare congenital disorder of neutrophil production due to impairment of myeloid differentiation in the bone marrow, with the neutrophil count being characteristically less than 500 x 10(3) cells/l (normal: 2-7 X 10(9)/l). Severe persistent neutropenia results in an increased susceptibility to frequent bacterial infections. The condition can be treated with recombinant human granulocyte colony-stimulating factor (G-CSF). Although several articles have addressed the clinicopathological and haematological aspects of this disorder, little or no information has been available concerning the radiological findings in this disorder. This report summarizes the clinical features, radiological findings and management of a patient with Kostmann's syndrome. (c) 2005 Elsevier Inc. All rights reserved.
Keywords
Kostmann's syndrome, Neutrophil, G-CSF
Divisions
fac_med
Publication Title
Clinical Imaging
Volume
29
Issue
5
Additional Information
Joazlina, ZY Wastie, ML Kamarulzaman, A