Epidemiology of congenital neutropenia.
Epidemiologic investigations of congenital neutropenia aim to determine several important indicators related to the disease, such as incidence at birth, prevalence, and outcome in the population, including the rate of severe infections, leukemia, and survival. Genetic diagnosis is an important criterion for classifying patients and reliably determining the epidemiologic indicators. Patient registries were developed in the 1990s. The prevalence today is probably more than 10 cases per million inhabitants. The rate of infection and leukemia risk can now be calculated. Risk factors for leukemia seem to depend on both the genetic background and cumulative dose of granulocyte colony stimulating factor.
Service d'Hémato Oncologie Pédiatrique Registre des neutropénies congénitales, Assistance Publique-Hôpitaux de Paris, Hopital Trousseau 26 Avenue du Dr Netter, Paris F 75012, France. jean.donadieu@trs.aphp.fr
Jean
J
Donadieu
Blandine
B
Beaupain
Nizar
N
Mahlaoui
Christine
C
Bellanné-Chantelot
D002471:Cell Transformation, Neoplastic; D006801:Humans; D015994:Incidence; D009017:Morbidity; D009503:Neutropenia / Q000151:congenital* / Q000453:epidemiology*; D015995:Prevalence; D011379:Prognosis; D012042:Registries; D012307:Risk Factors