- Thomas D Coates, MD
Thomas D Coates, MD
- Professor of Pediatrics and Pathology
- University of Southern California School of Medicine
This topic review discusses cyclic neutropenia (also called cyclic hematopoiesis), which, in contrast to other rare congenital neutropenias, tends to be mild and benign, but can occasionally be severe.
Other causes of neutropenia are discussed separately. (See "Overview of neutropenia in children and adolescents" and "Congenital neutropenia" and "Approach to the adult with unexplained neutropenia".)
Cyclic neutropenia is a rare, autosomal dominantly inherited disorder with variable expression and an estimated incidence of one to two per million [1,2]. It is characterized by neutropenia that recurs every 14 to 35 days, although over 90 percent of patients exhibit a cycle period of 21 days . While the disease tends to be benign, several affected patients have died of infection . Most of the patients present in the first year of life.
Rarely, patients with Shwachman-Diamond syndrome (SDS) can present with intermittent neutropenia that may mimic cyclic neutropenia, though the presentation may not be typical, and patients with cyclic neutropenia do not have the other dysmorphic features seen in SDS. (See "Shwachman-Diamond syndrome".)
There is an acquired adult onset form of cyclic neutropenia; however, it is autoimmune rather than genetic in nature and has been found in association with T cell large granular lymphocyte leukemia. (See "Clinical manifestations, pathologic features, and diagnosis of T cell large granular lymphocyte leukemia", section on 'Clinical features'.)
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