U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Constitutional neutropenia

MedGen UID:
1785816
•Concept ID:
C3805116
•
Disease or Syndrome
Synonyms: Benign ethnic neutropenia; Benign Ethnic Neutropenia; congenital neutropenia; constitutional neutropenia; genetic infantile agranulocytosis; infantile genetic agranulocytosis
SNOMED CT: Constitutional neutropenia (1156300000); Benign ethnic neutropenia (1156300000)
 
Monarch Initiative: MONDO:0015134
Orphanet: ORPHA101987

Definition

Chronic neutropenia in the absence of secondary causes, other cytopenia, splenomegaly, lymphadenopathy, or increased risk of infection. It occurs more frequently in individuals of African, Middle Eastern, and West Indian descent. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVConstitutional neutropenia

Professional guidelines

PubMed

Management of neutropenia in patients with rheumatoid arthritis.
Lazaro E, Morel J
Joint Bone Spine 2015 Jul;82(4):235-9. Epub 2015 Mar 26 doi: 10.1016/j.jbspin.2015.01.005. PMID: 25819216

Recent clinical studies

Etiology

Effect of circadian variation on neutrophil mobilization to the peripheral blood in benign constitutional neutropenia.
Souto Filho JTD, Portugal RD, Nucci M
Exp Hematol 2019 Jan;69:22-26. Epub 2018 Oct 25 doi: 10.1016/j.exphem.2018.10.007. PMID: 30367920
Management of neutropenia in patients with rheumatoid arthritis.
Lazaro E, Morel J
Joint Bone Spine 2015 Jul;82(4):235-9. Epub 2015 Mar 26 doi: 10.1016/j.jbspin.2015.01.005. PMID: 25819216
Neutropenia during combination therapy of interferon alfa and ribavirin for chronic hepatitis C.
Soza A, Everhart JE, Ghany MG, Doo E, Heller T, Promrat K, Park Y, Liang TJ, Hoofnagle JH
Hepatology 2002 Nov;36(5):1273-9. doi: 10.1053/jhep.2002.36502. PMID: 12395340
Leukocytes and the risk of ischemic diseases.
Ernst E, Hammerschmidt DE, Bagge U, Matrai A, Dormandy JA
JAMA 1987 May 1;257(17):2318-24. PMID: 3553628

Therapy

Safety and efficacy of an interleukin 12/23 inhibitor in a patient with constitutional neutropenia and psoriasis vulgaris.
Starek JV, Bechara CSR, Dultra MRER, Krakheche JMF
An Bras Dermatol 2021 Nov-Dec;96(6):795-797. Epub 2021 Sep 17 doi: 10.1016/j.abd.2020.11.010. PMID: 34544636Free PMC Article
Effect of circadian variation on neutrophil mobilization to the peripheral blood in benign constitutional neutropenia.
Souto Filho JTD, Portugal RD, Nucci M
Exp Hematol 2019 Jan;69:22-26. Epub 2018 Oct 25 doi: 10.1016/j.exphem.2018.10.007. PMID: 30367920
Neutropenia during combination therapy of interferon alfa and ribavirin for chronic hepatitis C.
Soza A, Everhart JE, Ghany MG, Doo E, Heller T, Promrat K, Park Y, Liang TJ, Hoofnagle JH
Hepatology 2002 Nov;36(5):1273-9. doi: 10.1053/jhep.2002.36502. PMID: 12395340

Prognosis

Leukocytes and the risk of ischemic diseases.
Ernst E, Hammerschmidt DE, Bagge U, Matrai A, Dormandy JA
JAMA 1987 May 1;257(17):2318-24. PMID: 3553628

Clinical prediction guides

Effect of circadian variation on neutrophil mobilization to the peripheral blood in benign constitutional neutropenia.
Souto Filho JTD, Portugal RD, Nucci M
Exp Hematol 2019 Jan;69:22-26. Epub 2018 Oct 25 doi: 10.1016/j.exphem.2018.10.007. PMID: 30367920
Leukocytes and the risk of ischemic diseases.
Ernst E, Hammerschmidt DE, Bagge U, Matrai A, Dormandy JA
JAMA 1987 May 1;257(17):2318-24. PMID: 3553628

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...