Chapter 48 – Autoimmune Neutropenia

In 1926, Charles Doan observed that sera from some patients agglutinated leukocytes from other individuals. This was the beginning of modern leukocyte immunology. The first report on autoimmune leucopenia was that of Francke, who attributed the disorder to the “leukolysine” he found in a patient’s serum. The efforts of an international cooperative research, initiated in 1964, led to the discovery of the HLA, the antigens that are expressed on lymphocytes and many tissues and are markers for histocompatibility. The finding of neutrophil-specific antigens suggested that other nucleated cells may also express “organ-specific” antigens. Autoimmune neutropenia was then established as a definable clinical entity when neutrophil-specific antigens were demonstrated to be a target in primary autoimmune neutropenias. More recently, the structure, genetics, and functions of neutrophil-specific antigens were determined and the finding that NA is a receptor for IgG was helpful in better understanding its clinical implications. Finally, the availability of colony-stimulating factors for treatment has had a major impact on the management of all neutropenias.