The Duffy antigen receptor for chemokines, ACKR1,– ‘Jeanne DARC’ of benign neutropenia

Naama Rappoport, Amos J. Simon, Ninette Amariglio, Gideon Rechavi

Research output: Contribution to journalReview articlepeer-review

23 Scopus citations


Benign neutropenia, observed in different ethnic groups, is the most common form of neutropenia worldwide. A specific single nucleotide polymorphism, rs2814778, located at the promoter of the ACKR1 (previously termed DARC) gene, which disrupts a binding site for the GATA1 erythroid transcription factor, resulting in a ACKR1-null phenotype, was found to serve as a predictor of low white blood cell and neutrophil counts in African-Americans and Yemenite Jews. Individuals with benign neutropenia due to the ACKR1-null allele have been found to have an increased susceptibility to human immunodeficiency virus infection and, on the other hand, a protective effect against malaria. The associated protective effect may explain the spread of the ACKR1-null allele by natural selection. The reviewed relationships between ACKR1 polymorphism and various pathological states may have important clinical implications to individuals with and without benign neutropenia. Potential mechanisms for ACKR1 (previously termed DARC) modulation during neutrophil recruitment to inflammation, and chemokine bioavailability in the circulation and in local tissue are reviewed and discussed.

Original languageEnglish
Pages (from-to)497-507
Number of pages11
JournalBritish Journal of Haematology
Issue number4
Early online date27 Dec 2018
StatePublished - Feb 2019

Bibliographical note

Funding Information:
GR holds the Djerassi Chair for Oncology (Sackler Faculty of Medicine, Tel Aviv University, Israel). This work was supported by the Varda and Boaz Dotan Research Center in Hemato-oncology affiliated to the Tel Aviv University.

Publisher Copyright:
© 2018 British Society for Haematology and John Wiley & Sons Ltd


  • ACKR1 polymorphism
  • Duffy null
  • FY allele
  • benign neutropenia
  • rs2814778


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