Epidemiology of congenital neutropenia

Jean Donadieu; Blandine Beaupain; Nizar Mahlaoui; Christine Bellanné-Chantelot

doi:10.1016/j.hoc.2012.11.003

Epidemiology of congenital neutropenia

Hematol Oncol Clin North Am. 2013 Feb;27(1):1-17, vii. doi: 10.1016/j.hoc.2012.11.003.

Authors

Jean Donadieu¹, Blandine Beaupain, Nizar Mahlaoui, Christine Bellanné-Chantelot

Affiliation

¹ 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

PMID: 23351985
DOI: 10.1016/j.hoc.2012.11.003

Abstract

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.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Cell Transformation, Neoplastic
Humans
Incidence
Morbidity
Neutropenia / congenital*
Neutropenia / epidemiology*
Prevalence
Prognosis
Registries
Risk Factors