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Neutropenia (Severe Chronic)

Background

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 cell 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 cause);
  • Cyclical Neutropenia is a form of SCN, with neutropenia occurring over a period of about three to seven days in a cycle of twenty-one 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.

What are the symptoms? View What are the symptoms?

Medical text written October 2005 by Contact a Family. Approved October 2005 by Dr P Ancliffe, Consultant Haematologist, Great Ormond Street Hospital, London, UK.

 

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