Abstract
Background and objectives: Severe chronic neutropenia (SCN) is a condition in which absolute neutrophil counts remain at a low level (under 500/µL) over months or years. Because of the rare onset of SCN, its epidemiology, prognosis, and clinical manifestations have not yet been fully understood. In particular, large-cohort studies in Asian countries are still insufficient. Therefore, in this study, national health insurance data was used to investigate the epidemiologic features and prognosis of SCN in South Korea. Materials and Methods: The data from the Health Insurance Review and Assessment database recorded between 1 January 2011 and 31 December 2015 were explored. SCN was defined based on the ICD-10 code, registry of benefit extension policy, and inclusion criteria of the study. After identifying patients with SCN, annual incidence and their co-morbidities were analyzed. Results: Among the initially identified patients with severe neutropenia (N = 2145), a total of 367 patients had SCN and were enrolled. The annual incidence rate of SCN ranged from 0.12 to 0.17 per 100,000 person-year (PY) during the study period. The highest incidence was observed in pediatric patients aged between 0 to 9 years (N = 156), followed by women in their fifties (N = 43). The total incidence rate was 0.17 in females and 0.12 in males (Relative risk (RR): 1.43, 95%, CI: 1.16–1.76). The most common accompanying condition was mild respiratory infection, but about 3.2% of patients progressed to hematologic malignancy after an average of 2.4 years. Conclusions: This nationwide population-based epidemiological study showed that incidence of SCN is higher in pediatrics and middle-aged women. As progression to hematologic malignancy was significantly higher in the age of in 45–49 years old, careful follow-up is necessary in this group. However, since this study lacks the molecular information, these finding need to be interpreted with great caution.
Highlights
Severe chronic neutropenia (SCN) is a condition in which absolute neutrophil counts remain under500/μL for months or years
The mean age at onset was 31.4 years; SCN occurred in 151 males (41.1%) and 216 females (58.9%)
This is the first study of the epidemiologic aspects of severe and chronic neutropenia that included the entire population of South Korea
Summary
Severe chronic neutropenia (SCN) is a condition in which absolute neutrophil counts remain under. This acute form of severe primary neutropenia is caused by cytotoxic drugs or infectious agents, whereas chronic forms arise from congenital, idiopathic, and autoimmune factors [1,2]. Due to the low incidence of SCN, its epidemiology, prognosis, and clinical manifestations have not yet been fully understood. Severe chronic neutropenia (SCN) is a condition in which absolute neutrophil counts remain at a low level (under 500/μL) over months or years. In this study, national health insurance data was used to investigate the epidemiologic features and prognosis of SCN in South Korea. The annual incidence rate of SCN ranged from 0.12 to 0.17 per 100,000 person-year (PY) during the study period. The total incidence rate was 0.17 in females and 0.12 in males (Relative risk (RR): 1.43, 95%, CI: 1.16–1.76)
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