Neutrophil disorders and their management

Abstract

Neutrophil disorders are an uncommon yet important cause of morbidity and mortality in infants and children. This article is an overview of these conditions, with emphasis on clinical recognition, rational investigation, and treatment. A comprehensive list of references is provided for further reading. (J Clin Pathol 2001;54:7-19)

Hypoxic regulation of neutrophil function and consequences for Staphylococcus aureus infection

“…The critical neutrophil concentration, where bacteria multiply and are phagocytosed at the same rate, has been determined for S. aureus as 400,000 neutrophils/ml [78], similar to the clinically relevant concentration of 500,000 neutrophils/ml, below which neutropaenic patients are at high risk of severe pyogenic bacterial infection. Furthermore, patients with severe congenital neutropaenia, leucocyte adhesion deficiency (impaired endothelial transmigration), ChediakHigashi syndrome (impaired chemotaxis and degranulation) and chronic granulomatous disease (defective ROS production), all suffer from recurrent staphylococcal infections [79].…”

Leucocyte adhesion deficiency type 1 with secondary haemophagocytic lymphohistiocytosis

“…The severity is based on the relative expression of CD18, with less than 1% of normal described as severe and 2.5-10% of normal as moderate. In the variable form CD18 levels are normal but non functional 6 .…”

Chediak–Higashi Syndrome Presented as Accelerated Phase: Case Report and Review of the Literature

“…The gene responsible for this defect was characterized in 1996 as the lysosomal trafficking regulator LYST or CHS1 gene located on chromosome-1 (1q42-43) [8,9]. Recently, 26 mutations in the CHS1 gene have been described, including nonsense and missense mutations, deletions and insertions [10,11]. This gene effects protein synthesis or/and maintenance of storage and secretory lysosomal granules of leukocytes and fibroblasts, dense bodies of platelets, azurophilic granules of neutrophils and melanosomes of melanocytes.…”

“…Lymphocytes contain large giant cytoplasmic granules and function poorly in antibody dependent cell mediated cytolysis of tumour cells. Natural killer cell function was also reduced [3,10,12]. A deficiency of granules containing serotonin and adenosine phosphate in platelets leads to defective platelet aggregation and prolonged bleeding time [5].…”