Neutropenia Information.....September 12, 2008

Neutropenia Information I've been getting a lot of questions about Brock's possible diagnosis of Cyclic Neutropenia, so I thought I would post some information here that we have been given (or referred to). Diagnosis and Testing Often, diagnosis of Neutropenia is difficult. Since it's a very rare condition, its symptoms are frequently assumed and confused with other conditions or diseases. With Cyclic Neutropenia counts can test normal due to a cyclical change, but may test very low at other times. Testing for Cyclic usually involves CBCs taken three times per week for at least six weeks to see if a regular cyclical pattern of neutrophil counts. Bone Marrow Testing If initial blood tests indicate Neutropenia the next step in diagnosis is a bone marrow examination. Neutropenia can be determined by viewing a sample of marrow under a microscope. Bone marrow cells are usually taken from the large pelvic bone, the ilium or the sternum. The patient is usually asleep under general anesthetic or under local anesthetic with sedation. treatment GRANULOCYTE-COLONY STIMULATING FACTOR (G-CSF) G-CSF is a cytokine normally produced by the human body itself. Cytokines are proteins and peptides that allow cells to communicate with one another. They act like hormones and neurotransmitters and are involved in a variety of immunological, inflammatory, and infectious diseases. The type of G-CSF which is given as treatment is not from human beings but is safely made by genetic engineering to produce an identical substance which acts like the naturally occurring cytokine. For most patients, 5-20 micrograms (mcg) per kilogram (kg) of body weight of G-CSF given as a daily subcutaneous injection is usually sufficient. Sometimes certain patients need very high doses, even up to 120/mcg/kg/day and others will require very low doses, as low as 0.01 mcg/kg/day. G-CSF treatments can be expensive, up to $4000 a month. OTHER TREATMENTS Bone Marrow Transplant BMT is a treatment option for SCN. It may be considered for failure to respond to treatment, or for patients who develop leukemia or MDS in the course of their disease. BMT is a very intensive procedure, carrying serious risks and therefore it is not recommended as first choice treatment. Your physician will be able to discuss this with you. Corticosteroids In some conditions steroids have long been effective at increasing neutrophil counts in the blood. Steroids work by encouraging neutrophils to leave the bone marrow and enter the blood stream. However, they do not induce the production of new neutrophils in the bone marrow. They may even decrease the number of other types of white cells increasing the risk of infection. (This information was from www.neutropenianet.org and was provided by our doctor.) Yikes! That is a lot of information, but I hope it answers some of the questions I have received. After next week, the diagnosis should be firm, but we may have to undergo a lot more testing before that happens.