소아청소년기에 발생한 중증 호중구감소증의 관찰 = Clinical and Laboratory Features of Severe Neutropenia Developed in Childhood

다국어 초록 (Multilingual Abstract)

Background: Neutropenia is not uncommon in children. We performed this study to investigate the etiology, clinical course and laboratory characteristics for prediction of recovery in children with severe neutropenia.
Methods: In this study, we studied the clinical course and hematological features of 107 patients with severe neutropenia who were diagnosed and treated at the Department of Pediatrics, The Catholic University of Korea from April 2009 to July 2014. Patients with hematologic disorders and malignant disease were excluded. Chronic severe neutropenia (CSN) was defined as an absolute neutrophil count of 0.5×109/L or less for at least 3 months. Acute severe neutropenia (ASN) were defined who recovered within 3 months from diagnosis.
Results: Among 107 patients, 72 patients showed ASN and 35 patients were CSN. Median age of ASN (15.2 months) was higher than that of CSN (10.7 months). The median duration of recovery from neutropenia was 7 days (range: 2-35) in ASN. ASN was commonly related to infectious diseases and 24 cases (33.3%) had documented etiologic agents. Median duration of recovery from neutropenia was 18 months in CSN. Three of 35 patients in CSN had ELANE gene mutation. The number of white blood cells, platelets, monocytes, and eosinophils were significantly higher in group of CSN compared with ASN (P＜0.05).
Conclusion: We confirmed the great etiological heterogeneity of severe neutropenia in this study. The kinetics of recovery from neutropenia was different between the ASN and CSN group and the complete blood counts may be useful indices for discriminating ASN from CSN.

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