Cyclic Neutropenia

I have a 14-month-old son who was just diagnosed with cyclic neutropenia. We were told by our pediatrician that it is inherited. I did some reading and found that usually the disorder is present in many family members. Nowhere in either my own or my husband's family can we find evidence of this disorder. I was also told that in some cases it can be a precursor to leukemia. Anything that you can tell me about this disorder would be of great help. Thanks.

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Robert Steele

Robert W. Steele, MD, is a board certified pediatrician at St. John's Regional Health Center in Springfield, MO. He graduated from medical... Read more

The neutrophils (NOO-tro-fills) are the types of white blood cells that are primarily responsible for gobbling up invading bacteria and other infectious organisms. Cyclic neutropenia is a disorder in which the number of these special cells drops dramatically in a cyclical pattern -- usually about every 21 days.

Typically, children with this disorder will have a normal number of these cells, then they will drop to extremely low levels lasting for about a week, and then rise again. During the time of a low neutrophil count, children can experience symptoms which include fever without an obvious cause, ulcers of the mouth, sore throat, enlarged lymph nodes, skin infections, and even more serious infections due to this important part of the immune system being limited.

How this disease arises is not entirely understood, but between 25 to 34 percent can be shown to due to inheritance. The remainder more than likely have some inherited basis for their disease, but it is often difficult to determine this. The diagnosis of cyclic neutropenia is often quite difficult because the symptoms may be quite variable in different children ranging from the rather mild in those whose numbers drop only moderately to the very severe in those whose neutrophil numbers drop to zero. This, coupled with the fact that these symptoms occur only during the low phase, makes cyclical neutropenia a difficult diagnosis to make.

Most of the time the child's examination is completely normal, so when cyclical neutropenia is considered, the only way to prove it is to have blood drawn twice per week for six to eight weeks. This allows tracking of the neutrophil numbers through two cycles. And even after all this blood is drawn, often blood from the bone marrow must also be obtained to rule out more serious conditions such a leukemia.

Management of this rare disorder involves treating the infections that arise and giving medications that can induce the bone marrow to produce more neutrophils. These medications are given as shots and go by the not-so- memorable names of G-CSF and GM-CSF. Most of the time, these shots do not completely stop the ups and downs of neutrophil counts, but it makes the low points not so low and last not so long.

Not all children with cyclic neutropenia inherit this disorder but certainly a good proportion of them do which makes examination of family members important in both making the diagnosis in the child as well as in other family members who might otherwise not know they have it. Finally, there is no evidence to suggest children with cyclic neutropenia are at any greater risk for leukemia.

I hope this helps.

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