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Primary neutropenia is defined as an absolute neutrophil count of ≤0.5 x 109/L in the absence of any known etiology. It is infrequently encountered, and there have been few studies of its natural history.
To elucidate the features of primary neutropenia, investigators analyzed data recorded in the French Severe Chronic Neutropenia Registry on 108 patients who were followed for a median of 8.3 years. They observed the following:
The ratio of women to men was 3.7, and the median age at diagnosis was 28.3 years.
26% of patients had very severe neutropenia (<0.2 x 109/L).
Bone marrow examination showed granulocytic hypoplasia in 14.8% of patients, maturation arrest in 30.6%, increased cellularity in 20.4%, and normal results in 34.3%. Hypoplasia wa…