PEDIATRICS Vol. 79 No. 5 May 1987, pp. 728-733
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Natural History of Primary Autoimmune Neutropenia in Infancy

Lars T. Conway MD1, Mary E. Clay MT(ASCP)1, William E. Kline MT, MS(ASCP)SBB1, Norma K. C. Ramsay MD1, William Krivit MD, PhD1, and Jeffrey McCullough MD1

1 From the Departments of Laboratory Medicine and Pathology and Pediatrics, University of Minnesota, Minneapolis, and the American Red Cross Blood Services, St Paul

Five patients with primary autoimmune neutropenia were evaluated during their first 2 years of life. Their illness resolved spontaneously after 6 to 41 months (median 13 months), and the patients were subsequently followed for 13 to 73 months (median 28 months). None required immunosuppressive therapy to induce remission, and routine antibiotic therapy adequately controlled all infectious episodes. An increased rate of infection, particularly otitis media and upper respiratory tract infection, occurred during the neutropenic period. No other noninfectious illnesses, particularly no other autoimmune diseases, were reported in any of these patients at any time. In each case, resolution of neutropenia paralleled the disappearance of neutrophil autoantibodies which were specific for the NA1 antigen. This report describes the clinical and laboratory findings and the long-term history of primary autoimmune neutropenia in these five patients.

Key Words: primary autoimmune neutropenia • infant • neutrophil • leukocyte antigen/antibody

Submitted on January 21, 1986
Accepted on June 10, 1986




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