TY - JOUR
T1 - Prevalence, phenotype and inheritance of benign neutropenia in Arabs
AU - Denic, Srdjan
AU - Showqi, Saad
AU - Klein, Christoph
AU - Takala, Mohamed
AU - Nagelkerke, Nicolaas Jan
AU - Agarwal, Mukesh Mansha
N1 - Funding Information:
This study was supported by grant no. 07/123 from the Faculty of Medicine and Health Sciences, UAE University, Al Ain, UAE. We acknowledge kind help with data collection of Mariam Al Sheikh, Wafa Nasar and Huda Abdul Ghani, Al Ain Hospital, Al Ain, UAE.
PY - 2009/3/27
Y1 - 2009/3/27
N2 - Background: Benign neutropenia, i.e., neutropenia not associated with an increased risk of infection, may result in serious medical consequences when a 'standard' definition of neutropenia (absolute neutrophil count (ANC) < 1.5 × 109 cells/L) is universally applied to all races. The aims of this study were to determine the prevalence of benign neutropenia among healthy Arabs and evaluate its mode of inheritance. Methods: ANCs were studied prospectively amongst a healthy indigenous population (n = 1032) from the United Arab Emirates undergoing a nation-wide sickle-cell and thalassemia screening program. The mean neutrophil count and the prevalence of benign neutropenia were compared by age, sex and amongst various tribes. Results: The mean neutrophil count (× 109 cells/L) was 3.3 (range 0.95-7.6). Benign neutropenia was present in 110 (10.7%) subjects of whom 24 (2.3%) individuals had moderate neutropenia (ANC 0.5 - 1.0 × 109 cells/L). In the 22 tribe-family groups, the prevalence of benign neutropenia varied between 0% and 38%. Benign neutropenia showed no difference in the frequency amongst the sexes (p = 0.23) and it was independent of age (Spearman's rho = 0.05, p = 0.13). The age-related mean neutrophil count was the lowest in Arabs when compared with other ethnic groups (Blacks, Europeans and Mexicans). The inheritance of benign neutropenia was consistent with an autosomal dominant pattern; however, the diversity of observed phenotypes suggested the presence of more than one genetic variant for this trait. Conclusion: Arabs have ahigh prevalence of benign neutropenia that may be inherited as an autosomal dominant trait.
AB - Background: Benign neutropenia, i.e., neutropenia not associated with an increased risk of infection, may result in serious medical consequences when a 'standard' definition of neutropenia (absolute neutrophil count (ANC) < 1.5 × 109 cells/L) is universally applied to all races. The aims of this study were to determine the prevalence of benign neutropenia among healthy Arabs and evaluate its mode of inheritance. Methods: ANCs were studied prospectively amongst a healthy indigenous population (n = 1032) from the United Arab Emirates undergoing a nation-wide sickle-cell and thalassemia screening program. The mean neutrophil count and the prevalence of benign neutropenia were compared by age, sex and amongst various tribes. Results: The mean neutrophil count (× 109 cells/L) was 3.3 (range 0.95-7.6). Benign neutropenia was present in 110 (10.7%) subjects of whom 24 (2.3%) individuals had moderate neutropenia (ANC 0.5 - 1.0 × 109 cells/L). In the 22 tribe-family groups, the prevalence of benign neutropenia varied between 0% and 38%. Benign neutropenia showed no difference in the frequency amongst the sexes (p = 0.23) and it was independent of age (Spearman's rho = 0.05, p = 0.13). The age-related mean neutrophil count was the lowest in Arabs when compared with other ethnic groups (Blacks, Europeans and Mexicans). The inheritance of benign neutropenia was consistent with an autosomal dominant pattern; however, the diversity of observed phenotypes suggested the presence of more than one genetic variant for this trait. Conclusion: Arabs have ahigh prevalence of benign neutropenia that may be inherited as an autosomal dominant trait.
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U2 - 10.1186/1471-2326-9-3
DO - 10.1186/1471-2326-9-3
M3 - Article
C2 - 19323844
AN - SCOPUS:65349184113
SN - 1471-2326
VL - 9
JO - BMC Blood Disorders
JF - BMC Blood Disorders
M1 - 3
ER -