Cyclic neutropenia is a rare cause of periodic fevers that occur during periodic drops in the absolute neutrophil count.
Inheritance: autosomal dominant (associated with a mutation at the elastase gene ELA2 on chromosome 19)
Onset is typically during infancy but the patient may present as an adult.
(1) cyclic drop in absolute neutrophil count (usually every 21 days, range 14 to 35 days)
(2) minimum absolute neutrophil count <= 200 per µL during an episode (some may use <= 300 per µL)
(3) concurrent bacterial infections during an episode, resulting in periodic fevers (often gingivitis or mucosal infections)
(1) The condition tends to become less severe as the patient gets older.
(2) Most patients have a period of 3-5 days while the absolute neutrophil count is at a minimum.
(3) Aphthous ulcerations may occur.
(1) failure to monitor absolute neutrophil count frequently enough to detect a transient drop in the count (recommended testing is 3 times a week for at least 8 weeks, depending on cycle duration)
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Specialty: Hematology Oncology, Clinical Laboratory