There are four main groups of severe chronic neutropenia (SCN).
Neutrophils are the most numerous white cells in the blood. In SCN,
individuals have below normal numbers of neutrophils, the white
blood cells which combat bacterial infections. The lack of
neutrophils results in the individual having an impaired ability to
fight infection. SCN affects people of both sexes and all ethnic
groups with a possible predominance in Caucasian people. SCN is
rare with an incidence thought to be about 1 to 2 in 1,000,000.
SCN types are:
- congenital neutropenia in which diagnosis is usually made soon
after birth. Congenital neutropenia can be inherited or sporadic
(with no other affected family members but still of genetic
- cyclical neutropenia is a form of SCN, with neutropenia
occurring over a period of about three to seven days in a cycle of
21 days. Cyclical neutropenia is often caused by mutations of the
ELA2 gene on chromosome 19
- idiopathic neutropenia is the name given to children and adults
described as neutropenic in which no clear cause can be found.
Affected people may have had a normal blood cell count in the past.
Idiopathic neutropenia is usually a relatively mild condition
- autoimmune neutropenia is most common in infants and young
children. The body identifies the neutrophils as enemies and makes
antibodies to destroy them. Children usually grow out of it within
two years of diagnosis.
Medical text written October 2005 by Contact a Family. Approved
October 2005 by Dr P Ancliff, Consultant Haematologist, Great
Ormond Street Hospital, London, UK.