Adult-onset cyclic neutropenia is associated with increased large granular
lymphocytes
TP Loughran , EA Clark, TH Price and WP Hammond
Human cyclic neutropenia occurs in adults as well as children. Clinical
illness is similar in the childhood and adult diseases, but distinctly
different modes of onset suggest heterogeneity in its pathophysiology. We
studied seven patients with cyclic neutropenia, three with disease acquired
in adulthood, and four with the childhood-onset disorder. All three
patients with adult-onset cyclic neutropenia had increased numbers of
circulating large granular lymphocytes (LGL), whereas the four children
with cyclic neutropenia had normal LGL counts. LGL from patients with
adult-onset cyclic neutropenia expressed cell surface antigens HNK-1 (three
of three patients) and IgG Fc receptors (two of three patients), although
natural killer activity was low. Two of these patients were treated with
alternate-day steroids, resulting in decreased LGL counts and abrogation of
neutrophil cycling. We suggest that adult-onset cyclic neutropenia may be
distinguished from the childhood-onset form of the disease by increased
numbers of LGL. Furthermore, increased LGL may identify a subset of
patients with cyclic neutropenia who respond to steroid therapy.
Volume 68,
Issue 5,
pp. 1082-1087,
11/01/1986
Copyright © 1986 by The American Society of Hematology
