Cyclic neutropenia

Cyclic neutropenia caused by defects in ELA2 is an autosomal dominant disease in which blood-cell production from the bone marrow oscillates with 21-day periodicity. Circulating neutrophils vary between almost normal numbers and zero. During intervals of neutropenia, affected individuals are at risk for opportunistic infection. Monocytes, platelets, lymphocytes and reticulocytes also cycle with the same frequency.

Alternative names

ELA2 related neutropenia

Cyclic haematopoiesis

Classification

  • Defects of phagocyte function
    • Severe congenital and cyclic neutropenias
      • Cyclic neutropenia

Inheritance

Autosomal dominant

OMIM

#162800 Cyclic hematopoiesis

*130130 Elastase 2; ELA2

Cross references

Phenotypically related immunodeficiencies

IDR factfile for Severe congenital neutropenias, including Kostmann syndrome

Incidence

1: 1,000,000