Statement regarding Neutropenia
Thursday 30 Oct 2008
Severe chronic neutropenia (SCN) is the name given to a group of conditions in which neutropenia is the primary problem. The severity in symptoms of the neutropenia differs widely among the various sub-types of neutropenia and even from patient to patient within each disease sub-type. The bone marrow is where all blood cell production takes place. The bone marrow is the site where three basic types of blood cells are produced. These include the red blood cells, which carry oxygen from the lungs to all tissues of the body; the platelets, which are essential for the clotting of blood and the white blood cells, which are in charge of the body’s defense against infection. There are three main types of white blood cells; granulocytes, monocytes, and lymphocytes. Neutrophils make up the major part of the granulocytes.
The term neutropenia describes the situation where the number of neutrophils in the blood is too low. Neutrophils are important in defending the body against bacterial infections and therefore, a patient with too few neutrophils is more susceptible to bacterial infection. Neutropenia can occur for different reasons. Patients with cancer may become neutropenic because of chemotherapy they receive. Sometimes neutropenia occurs after a viral infection. Some patients are born with neutropenia as in the case of Cohen Syndrome.
The level of neutropenia may vary considerably in general. The blood of healthy adults contains about 1,500 to 7,000/ml of neutrophils. In children under 6-years of age, the neutrophil count may be lower. The severity of neutropenia generally depends on the absolute neutrophil count (ANC) and is described as follows; severe neutropenia occurs when the ANC falls below 500/ml. If the patient has neutropenia for more than three months, the patient is described as suffering from chronic neutropenia.
Symptoms which patients experience depends the level of neutropenia. The lower the neutrophil count the greater the risk for infection. This risk increases if low neutrophil counts persist for more than three days. Types of infection include otitis media, tonsillitis, sore throats, mouth ulcers, gum infections and skin abscesses. Any fever must be taken seriously and your nurse or physician should be informed.
Severe neutropenia can lead to serious problems, which require prompt care and attention as the patient could potentially develop a bacterial fungal mixed infection at any time. These infections can be life threatening when the patient is persistently severe neutropenic and it is therefore important that if the patient develops any signs or symptoms of infection then he or she should be seen by a doctor as soon as possible and treated with medications to fight the infection.
Severe chronic neutropenia is associated with Cohen Syndrome and is fortunately amenable to treatment with Neupogen (granulocyte-colony-stimulating factor; G-CSF). Further information about the use of G-CSF can be found on the Severe Chronic Neutropenia International Registry (SCNIR) website.
This material was modified from the SCNIR patient handbook.