title: Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases.
abstract: Primary autoimmune neutropenia (AIN) is caused by granulocyte-specific autoantibodies and occurs predominantly in infancy. Clinical presentation and diagnosis have not been well established, resulting in burdening diagnostic investigations and unnecessary treatment with granulocyte colony-stimulating factor (G-CSF). In the present study, clinical, laboratory, and immunologic data of 240 infants with primary AIN were evaluated. Suspected association with parvovirus B19 infection was investigated using serologic and DNA-based methods. Primary AIN was mainly diagnosed at the age of 5 to 15 months but was observed as early as day 33 of life. In 90% of the cases, AIN was associated with benign infections despite severe neutropenia. Spontaneous remission, shown by 95% of the patients, usually occurred within 7 to 24 months. Autoantibodies in the patient's sera were not always present, and screening had to be repeated several times until antibody detection succeeded. About 35% of the autoantibodies showed preferential binding to granulocytes from NA1 and NA2 homozygous donors. Bone marrow was typically normocellular or hypercellular, with a variably diminished number of segmented granulocytes. A significant association with parvovirus B19 infection was not found. Symptomatic treatment with antibiotics was sufficient in most patients. Eighty-nine percent of the patients received antibiotics (cotrimoxazole) for prophylaxis of infections. For severe infections or for surgical preparation, G-CSF, corticosteroids, and intravenous IgG were administered, resulting in increased neutrophil counts in 100%, 75%, and 50% of the patients treated, respectively. In combination with the detection of granulocyte-specific antibodies, the typical clinical picture allowed diagnosis of AIN without burdening investigations. Treatment with G-CSF was found to be a reliable alternative to temporarily increase the neutrophil count.
authors.affiliation: Institute for Clinical Immunology and Transfusion Medicine, Justus-Liebig University, Giessen, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Bux
authors.forename: G
authors.initials: G
authors.lastname: Behrens
authors.forename: G
authors.initials: G
authors.lastname: Jaeger
authors.forename: K
authors.initials: K
authors.lastname: Welte
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000914:Antibodies, Viral / Q000097:blood; D000918:Antibody Specificity; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000628:therapy / Q000821:virology; D001424:Bacterial Infections / Q000150:complications / Q000188:drug therapy; D001853:Bone Marrow / Q000473:pathology; D002675:Child, Preschool; D004279:DNA, Viral / Q000097:blood; D018450:Disease Progression; D005260:Female; D000069585:Filgrastim; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008297:Male; D009503:Neutropenia / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000628:therapy / Q000821:virology; D009504:Neutrophils / Q000276:immunology; D010322:Parvoviridae Infections / Q000150:complications / Q000276:immunology; D016732:Parvovirus B19, Human / Q000276:immunology / Q000302:isolation & purification / Q000472:pathogenicity; D011994:Recombinant Proteins; D012075:Remission, Spontaneous
chemical_list: D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents; D000914:Antibodies, Viral; D001323:Autoantibodies; D001324:Autoantigens; D004279:DNA, Viral; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D000069585:Filgrastim