[Adult cyclic neutropenia: acute ileitis as an initial clinical manifestation]

Cyclic neutropenia is a rare disease in which blood neutrophils periodically decrease and almost disappear, ensuing acute infections of variable severity. We report a 37 years old female presenting with severe abdominal pain, fever and neutropenia. During a surgical abdominal exploration, an acute ileitis was found. During a follow-up of 6.5 months, transient episodes of neutropenia (mean count of 0.33 x 10(9)/l), every 22-28 days, were detected. Coinciding with these episodes, band and segmented neutrophils disappeared from the bone marrow and its response to functional stimulation with prednisone was insufficient. No cyclical changes in lymphocytes, monocytes and packed red cell volume were observed. The episodes of severe transient neutropenia and infections persist after three years of follow up.