DIAGNOSIS AND CLINICAL
COURSE OF AUTOIMMUNE NEUTROPENIA IN INFANCY: ANALYSIS OF 18 CASES
Taniuchi S, Tsuji S,
Hasui M, Yamamoto A, Kobayashi Y
Department of
Pediatrics, Kansai Medical University, Osaka, Japan
Objective: Autoimmune neutropenia
in infancy (AIN) is caused by granulocyte-specific autoantibodies and
occurs predominantly in childhood. Clinical presentation and diagnosis have
not been well studied, resulting in burdening diagnostic investigations and
unnecessary treatments such as granulocyte-colony stimulating factor
(G-CSF).
Methods: Clinical, laboratory,
and immunological data of 18 infants with AIN were evaluated.
Granulocyte-specific autoantibodies were detected by direct granulocyte
immunofluorescence鞍test (D-GIFT), indirect granulocyte immunofluorescence test(I-GIFT )and
immunoblotting .
Results: The average age of
onset and resolution of neutropenia in AIN were 7.4?.4 months(mean盨D) and 20.4?.9 months
(mean盨D), respectively. Sixteen of the 18 patients presented mild
infectious symptoms. The other 2 patients were detected by chance and
presented no infectious symptom. D-GIFT was positive in all patients, and
I-GIFT was positive in 17 of these 18 patients, respectively. Most patients
showed preferential binding to neutrophils from NA (1+2-) phenotyped donors
by I-GIFT and Immunoblotting. Fifteen patients received antibiotic
(sulfamethoxazole -trimetoprme) for prophylaxis. G-CSF was administrated in
only a patient in infectious state.
Conclusions: Combination of these 3
methods for the detection of granulocyte-specific autoantibody was useful
to make the diagnosis of AIN, resulting in alleviating diagnostic
investigations. Continuous treatment of G-CSF was unnecesary because of
treatment of ST for prophylaxis or, mild or chanced infection.