Table of ContentsView AllTable of ContentsDefinitionSymptomsCausesDiagnosisTreatmentPrognosis

Table of ContentsView All

View All

Table of Contents

Definition

Symptoms

Causes

Diagnosis

Treatment

Prognosis

This article discusses the causes and diagnostic options for benign ethnic neutropenia.

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Defining Benign Ethnic Neutropenia

Neutropenia is typically inherited or acquired and usually increases a person’s risk for infection, though this is not the case for people with BEN.

First described in 1941, approximately 4.5% of African-Americans have the condition, whereas less than 1% of Americans with European ancestry have BEN.

Benign Ethnic Neutropenia Symptoms

People with BEN do not have symptoms.

The diagnosis of BEN should only be made in people who have had repeat testing that shows persistent mild neutropenia, a neutrophil count between 1,000/mm3to 1,500/mm3, without any symptoms.

People with BEN should not have any of the following:

Scientists are not exactly sure why specific populations develop BEN. However, studies show a strong association with a genetic change on the DARC (Duffy antigen receptor for chemokines) gene located on chromosome 1.This genetic change is widespread in Africans and African-Americans and nearly non-existent in white Europeans.

The genetic change in DARC is also protective againstPlasmodium vivax, amalariaparasite.Since malaria is widespread in African nations, scientists believe that natural selection contributed to the DARC genetic change being more common in people of African or Middle Eastern descent.

Additionally, since the change in DARC is protective against malaria and is the same change associated with BEN, BEN is more prevalent in people of African and Middle Eastern descent.

Scientists are still unclear how the genetic change leads to neutropenia. Scientific studies support several different theories, which include:

However, further exploration is underway because scientists think that other factors must also be involved in the development of BEN.

BEN is a diagnosis of exclusion, meaning that a healthcare provider should eliminate all other causes of neutropenia first.

People with BEN have a laboratory test that shows a low neutrophil count of less than 1,500/mm3. In addition, they:

The performed test is called acomplete blood cell count, and the white blood cell and absolute neutrophil counts will consistently be a little low in three different samples. Repeat blood tests should be done at least two weeks apart.

Experts debate about the need for further testing, but there are some general recommendations:

These guidelines only apply to adults. In children, further testing is required even for mild neutropenia.

BEN in ChildrenAdditional tests are needed to diagnose BEN in children. However, extensive testing, particularly in children of African or Middle Eastern descent, is not recommended.This is especially true if a child’s parent has already been diagnosed with BEN.

BEN in Children

Additional tests are needed to diagnose BEN in children. However, extensive testing, particularly in children of African or Middle Eastern descent, is not recommended.This is especially true if a child’s parent has already been diagnosed with BEN.

BEN does not require treatment. It is a benign illness that does not lead to infections or other chronic problems.

However, a chronically low neutrophil count can affect cancer patients who are treated withchemotherapy.People with BEN should discuss with their oncologist or healthcare provider when deciding whether to reduce or stop chemo as a result of a low white blood cell count.

BEN does not increase a person’s risk for further health problems.

With data from the Centers for Disease Control and Prevention (CDC), experts found that people with BEN have:

Summary

A Word From Verywell

If you are of African or Middle Eastern descent and are told that you have a low white blood cell or neutrophil count, you may want to raise the possibility of BEN with your healthcare provider. If you do not have any other symptoms or health problems, you can consider repeat neutrophil count testing rather than other specialized testing and investigations, which can become expensive. Discuss the best diagnostic recommendations with your healthcare provider.

4 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Lakhotia R, Aggarwal A, Link ME, Rodgers GP, Hsieh MM.Natural history of benign ethnic neutropenia in individuals of African ancestry.Blood Cells Mol Dis. 2019;77:12-16. doi:10.1016/j.bcmd.2019.01.009Atallah-Yunes SA, Ready A, Newburger PE.Benign ethnic neutropenia.Blood Rev. 2019;37:100586. doi:10.1016/j.blre.2019.06.003Awan ZA, Al Amoudi SM, Saboor M, Alkhaldy HY.Isolated neutropenia/benign ethnic neutropenia: a common clinical and laboratory finding in southern and western saudi arabia.Int J Gen Med. 2021;14:451-457. doi:10.2147/IJGM.S300690Reich D, Nalls MA, Kao WH, et al.Reduced neutrophil count in people of African descent is due to a regulatory variant in the Duffy antigen receptor for chemokines gene.PLoS Genet. 2009;5(1):e1000360. doi:10.1371/journal.pgen.1000360

4 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Lakhotia R, Aggarwal A, Link ME, Rodgers GP, Hsieh MM.Natural history of benign ethnic neutropenia in individuals of African ancestry.Blood Cells Mol Dis. 2019;77:12-16. doi:10.1016/j.bcmd.2019.01.009Atallah-Yunes SA, Ready A, Newburger PE.Benign ethnic neutropenia.Blood Rev. 2019;37:100586. doi:10.1016/j.blre.2019.06.003Awan ZA, Al Amoudi SM, Saboor M, Alkhaldy HY.Isolated neutropenia/benign ethnic neutropenia: a common clinical and laboratory finding in southern and western saudi arabia.Int J Gen Med. 2021;14:451-457. doi:10.2147/IJGM.S300690Reich D, Nalls MA, Kao WH, et al.Reduced neutrophil count in people of African descent is due to a regulatory variant in the Duffy antigen receptor for chemokines gene.PLoS Genet. 2009;5(1):e1000360. doi:10.1371/journal.pgen.1000360

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

Lakhotia R, Aggarwal A, Link ME, Rodgers GP, Hsieh MM.Natural history of benign ethnic neutropenia in individuals of African ancestry.Blood Cells Mol Dis. 2019;77:12-16. doi:10.1016/j.bcmd.2019.01.009Atallah-Yunes SA, Ready A, Newburger PE.Benign ethnic neutropenia.Blood Rev. 2019;37:100586. doi:10.1016/j.blre.2019.06.003Awan ZA, Al Amoudi SM, Saboor M, Alkhaldy HY.Isolated neutropenia/benign ethnic neutropenia: a common clinical and laboratory finding in southern and western saudi arabia.Int J Gen Med. 2021;14:451-457. doi:10.2147/IJGM.S300690Reich D, Nalls MA, Kao WH, et al.Reduced neutrophil count in people of African descent is due to a regulatory variant in the Duffy antigen receptor for chemokines gene.PLoS Genet. 2009;5(1):e1000360. doi:10.1371/journal.pgen.1000360

Lakhotia R, Aggarwal A, Link ME, Rodgers GP, Hsieh MM.Natural history of benign ethnic neutropenia in individuals of African ancestry.Blood Cells Mol Dis. 2019;77:12-16. doi:10.1016/j.bcmd.2019.01.009

Atallah-Yunes SA, Ready A, Newburger PE.Benign ethnic neutropenia.Blood Rev. 2019;37:100586. doi:10.1016/j.blre.2019.06.003

Awan ZA, Al Amoudi SM, Saboor M, Alkhaldy HY.Isolated neutropenia/benign ethnic neutropenia: a common clinical and laboratory finding in southern and western saudi arabia.Int J Gen Med. 2021;14:451-457. doi:10.2147/IJGM.S300690

Reich D, Nalls MA, Kao WH, et al.Reduced neutrophil count in people of African descent is due to a regulatory variant in the Duffy antigen receptor for chemokines gene.PLoS Genet. 2009;5(1):e1000360. doi:10.1371/journal.pgen.1000360

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