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Neutrophil disorders can be grouped into deficiencies (neutropenia), excesses (neutrophilia), or qualitative abnormalities. Neutropenia can have the severe consequence of predisposing to infection, whereas neutrophilia usually is a manifestation of an underlying inflammatory or neoplastic disease, the neutrophilia, per se, having no specific consequences. Qualitative disorders of neutrophils may lead to infection as a result of defective cell translocation to an inflammatory site or defective microbial killing. Neutropenia may reflect an inherited disease that is evident in childhood (eg, congenital [hereditary] severe neutropenia), but more often, it is acquired. A common cause of neutropenia is the adverse effect of a drug. Some cases of neutropenia have no evident cause. The health consequence of neutropenia is a function of the mechanism of the neutropenia, the abruptness and severity of the decrease in the blood neutrophil count, and the duration of the decrease. Neutrophils have also been identified as mediators of vascular or tissue injury. Table 62–1 provides a comprehensive categorization of quantitative and qualitative neutrophil disorders.

Acronyms and Abbreviations

CD, cluster of differentiation; G-CSF, granulocyte colony-stimulating factor; HLA-DR, human leukocyte antigen-D related; NET, neutrophil extracellular traps.


Table 62–1 lists disorders that result from a primary abnormality in neutrophil numbers or function. Neutropenia or neutrophilia also occurs as part of a disorder that affects multiple blood cell lineages, as in infiltrative diseases of the marrow; intrinsic disorders of multipotential marrow hematopoietic cells; or removal of several blood cell types in the circulation. These diseases are not included in this classification and are discussed in other chapters of this text. This classification and chapter considers disorders in which the neutrophil either is the only cell type affected or the dominant cell type affected.

TABLE 62–1.Classification of Neutrophil Disorders

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