eISSN: 1644-4124
ISSN: 1426-3912
Central European Journal of Immunology
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4/2007
vol. 32
 
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abstract:


Clinical guidelines
Chronic neutropenia in children – diagnostics, therapeutic management and prophylaxis

Maja Klaudel-Dreszler
,
Ewa Bernatowska

(Centr Eur J Immunol 2007; 32 (4): 226-233)
Online publish date: 2007/12/10
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Chronic neutropenia (CN) is a rare disorder in children. It is defined by an absolute neutrophil count (ANC) below 1500 cells per cubic microliter of peripheral blood, lasting for at least 6 months. It can be acquired or congenital. More frequent – acquired chronic neutropenia is mainly caused by: malnutrition, exposure to drugs, chemical compounds and viral infections. CN is the cardinal symptom of some rare, hereditary entities such as: chronic benign familial neutropenia, Shwachman-Diamond syndrome (SDS), Kostmann syndrome (KS), cyclic neutropenia (CyN), glycogen storage disease type 1b (GSD1b). It may be also associated with primary immunodeficiency (PID), organic acidaemia or autoimmune disease. Diagnostics requires: detailed medical history of a child, repeated complete blood counts (CBS) including absolute neutrophil count (ANC), bone marrow aspirate, granulocytic antibodies – in cases of severe neutropenia, sometimes – immunologic investigation. Therapeutic management depends on the clinical entity, patient’s age and severity of clinical course; it includes antibiotic and vaccine prophylaxis, in selected children – chronic rHuG-CSF-treatment or haematopoietic stem cell transplantation (HSCT) from an HLA – identical related or unrelated donor, especially in children suffering from the diseases connected with relatively high risk of malignant myeloid transformation such as: KS, SDS and Fanconi anaemia (FA). Currently, autoimune neutropenia of infancy (AIN), a benign and self-limiting condition, is more often diagnosed by anti-neutrophil antibodies detection, in children under 5 years of age.
keywords:

chronic neutropenia, primary immunodeficiency, anti-granulocytic antibodies, rHuG-CSF, haematopoietic stem cell transplantation

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