Diagnosis and Clinical Course of Autoimmune Neutropenia in Infancy: Analysis of 240 Cases

摘要: Primary autoimmune neutropenia (AIN) is caused by granulocyte-specific autoantibodies and occurs predominantly in infancy. Clinical presentation diagnosis have not been well established, resulting burdening diagnostic investigations unnecessary treatment with granulocyte colony-stimulating factor (G-CSF). In the present study, clinical, laboratory, immunologic data of 240 infants primary AIN were evaluated. Suspected association parvovirus B19 infection was investigated using serologic DNA-based methods. mainly diagnosed at age 5 to 15 months but observed as early day 33 life. 90% cases, associated benign infections despite severe neutropenia. Spontaneous remission, shown 95% patients, usually occurred within 7 24 months. Autoantibodies patient's sera always present, screening had be repeated several times until antibody detection succeeded. About 35% showed preferential binding granulocytes from NA1 NA2 homozygous donors. Bone marrow typically normocellular or hypercellular, a variably diminished number segmented granulocytes. A significant found. Symptomatic antibiotics sufficient most patients. Eighty-nine percent patients received (cotrimoxazole) for prophylaxis infections. For surgical preparation, G-CSF, corticosteroids, intravenous IgG administered, increased neutrophil counts 100%, 75%, 50% treated, respectively. combination antibodies, typical clinical picture allowed without investigations. Treatment G-CSF found reliable alternative temporarily increase count.