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Hereditary neutrophilia

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Disease definition

A rare, genetic, immune disease characterized by chronic neutrophilia, increase in the percentage of circulating CD34+ cells in peripheral blood, increase in granulocyte precursors in bone marrow and splenomegaly. Patients are predominantly asymptomatic, but may present with systemic inflammatory response syndrome with fever, dyspnea, tachycardia, pleural and pericardial effusion, or myelodysplastic syndrome.

ORPHA:279943

Classification level: Disorder

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant

Age of onset: All ages

ICD-10: D72.8

OMIM: 162830

UMLS: C0543669

MeSH: C563010

A summary on this disease is available in Français (2019) Español (2019) Deutsch (2019) Italiano (2019) Nederlands (2019)
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