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Diagnosis and Clinical Course of Autoimmune Neutropenia in
Infancy: Analysis of 240 Cases
Juergen Bux,
Georg Behrens,
Gudrun Jaeger, and
Karl Welte
From the Institute for Clinical Immunology and Transfusion Medicine,
Justus-Liebig University, Giessen, Germany; the Max von Pettenkofer
Institute, Ludwig-Maximilians University, Munich, Germany; and the
Department of Pediatric Hematology and Oncology, Hannover Medical
School, Hannover, Germany.
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.
Blood, Vol. 91 No. 1 (January 1), 1998:
pp. 181-186
© 1998 by The American Society of Hematology.
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