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- Mon Dec 12, 2005 12:16 am
My son has a history of abnormal blood counts. His white count ranges from high to excessively low. His platelets from normal to extremely high and his red count is typically mildly low to the low end of normal. His doctor isnt concerned because the numbers don't stay consistently high or low. They always recheck in a few weeks and see a vast change. On his most recent test, his counts were all within normal range (for the first time ever), but there was a flag on the histiogram in the R1 region for lymphocytes. Can anyone tell me what might be the cause of this and if it is anything we should be concerned about?
| Dr. Tamer Fouad
- Wed Jan 25, 2006 6:41 am
Cyclic neutropenia is a rare congenital granulocytopoietic disorder. It may be inherited in an autosomal dominant fashion.
Cyclic neutropenia can occur sporadically, but there are families in which cyclic neutropenia is inherited with one parent and more than one child affected. They are usually diagnosed before the age of 10.
As the name indicates, in this disease neutrophil counts show irregular, periodic oscillations in the number of peripheral neutrophils with a typical cycle length of 21 ± 3 days. These cycles vary from patient to patient with some individuals being neutropenic during the whole cycle and others who have low neutrophil counts for only a few days and normal blood counts during the rest of the cycle.
The frequency of bacterial infections depends on the length of the neutropenic period that the patient experiences. Those who have a longer neutropenic period within the cycle suffer more frequently from infections compared to patients who have only short neutropenic phases.
Cyclic neutropenia is suspected if infections (typically aphthous stomatitis - inflammation and ulceration of the mouth) occur frequently in approximately 3-week intervals. A serial differential blood counts is needed (at least 3 times per week over six weeks) to search for the typical cyclical pattern of blood neutrophils in this disease.
Almost all patients with clinically obvious cyclic neutropenia have periods of severe neutropenia (ANC less than 200 cells/µl) (0.2 x 109/l) every 3 weeks showing some symptoms with almost every cycle, but significant infections (e.g. otitis media, pneumonia and bacteraemia) usually are infrequent.
Cyclic neutropenia occurs because of fluctuating rates of cell production by the bone marrow stem cells. In contrast to other causes for neutropenia, in this condition the marrow changes during the cycle, between normal appearance and that of severe maturation arrest of neutrophil production.
Other blood cells, such as platelets or red cells can also show oscillations with a cyclical pattern.
Glucocorticoids and G-CSF treatment prevent the episodic neutropenia and reduce infectious complications in some patients. Rigorous oral hygiene and judicious use of antibiotics at the nadir of the ANC are indicated. No predisposition to hematologic malignancies has been reported.
The underlying genetic defect of cyclic neutropenia has recently been discovered. This might lead to new therapeutical approaches for cyclic neutropenia patients in the future.
I would recommend you discuss this possibility with your doctor and to refer your son to a pediatric hematologist if he experiences cyclic infections.