ABSTRACT
Neutrophilic granulocytes, or simply neutrophils, are phagocytic cells that are an important part of the human body’s non-specific defense system. The cells are easily recognized in blood smears by their characteristic multilobed nuclei and the fine stippling throughout the cytoplasmic compartment, representing primary and secondary granules containing distinct sets of proinflammatory mediators. If the number of neutrophils in the body are significantly reduced or their function impaired, the individual becomes susceptible to repeated bacterial or fungal infections. Such neutrophil reduction, resulting in neutropenia, may be caused by an autoimmune mechanism, as detailed in this chapter, but may also be due to a variety of other disease conditions, including infections, malignant disease and congenital disorders. Many aspects of the disease mechanism of autoimmune neutropenia are poorly understood, although it seems clear that the antibodies, either as specific antibodies directed against surface molecules on neutrophils or their precursors, or as part of immune complex, are intimately involved in the peripheral destruction of neutrophils. Currently, a increasing effort is being undertaken to develop more standardized techniques for evaluation of autoimmune neutropenia. In this review the classification of autoimmune neutropenia, the underlying disease autoimmune mechanisms and the current therapeutic possibilities will be considered.
