Previously healthy white male 1 year 11 months old. History of 8 months of recurrent fever and intermittent neutropenia. Fevers come every 18 - 28 days. Testing for cyclic neutropenia initially showed neutropenia at anticipated time, fever as excepted then no further cycling during the following 6 weeks of study. Fevers are 103-105 and last 2-5 days usually with no other symptoms (occasionally diarrhea & rash, stomatitis & gingivitis) No history of otitis media or strep - when evaluated no cause of infection found. Immune studies normal, EBV studies negative, HIV negative, chest x-ray negative, phyiscal exam normal except presence of small pea sized nodules in lymph nodes under the chin. Mother has a history of MS - no other family history of note. Where do we go from here ?
Periodic fever syndromes are challenging. The case described clearly does not fit classic cyclic neutropenia although it may have at its inception. It appears that fever is currently cycling alone. The note does not state if leukopenia is still present, even though neutropenia is implied not to be. By any chance, has leukocyctosis been present more recently? I ask this because the syndrome of periodic fever, pharyngitis, and aphthous stomatitis, associated with elevated serum IGD, comes to mind. I realize that you stated immune studies were normal, but perhaps refocus on IgD would be worthwhile. A single dose of oral prednisone (2mg/kg) is reputed to curtail such episodes (see Pediatric Notes May 21, 1998, page 82). I wonder if a bone marrow has been considered to determine if any relationship to other forms of cyclic neutropenia exists. I assume there is no possibility of Munchhausen syndrome by proxy as an explanation. I hope these thoughts are of some use. They are presented for educational purposes only.
Key words: periodic fever, neutropenia
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