Cyclic Neutropenia ? Case Report

Cyclic neutropenia is a rare disorder characterized by a cyclic reduction in the granulocyte proliferative pool in the bone marrow followed by the onset of neutropenia. We report a male patient 79 years of age, with 33-year history of disease. Antineutrophil antibody investigations revealed that the indirect granulocyte immunofluorescence test was positive (IgM), suggesting the presence of granulocyte-specific IgM antibodies in the serum of the patient. The impression gained was of highly stimulated reactive immune system. Treatment started with G-CSF (and occasionally antibiotics) only in the periods when WBC count was less than 1, 5x109/L for several years, but lately the patient has been on weekly and the last year on twice weekly therapy with G-CSF, with normal WBC count and ANC varying from 110-3960, without any significant infection. The novel treatment like the CXCR4 antagonist plerixafor (Mozobil, Genzyme), shows promise in the way it raises blood counts, including both neutrophils and lymphocytes.