Cyclic Neutropenia — MFDS Part 1 MCQ
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Correct answer: A — Severe neutropenia recurring at approximately 21-day intervals, with recovery of the neutrophil count between episodes
Explanation lettering: E = shown as B · B = shown as C · C = shown as D · D = shown as E
Cyclic neutropenia causes regular oscillation of the absolute neutrophil count, typically with a severe nadir approximately every 21 days and recovery between episodes. Fever, painful oral ulceration and gingival or periodontal inflammation commonly coincide with the neutropenic phase. Persistent severe neutropenia without intervening recovery suggests severe congenital neutropenia (B). Persistent neutrophilia with impaired migration is characteristic of leucocyte adhesion deficiency (C), whereas a normal neutrophil count with defective intracellular killing suggests chronic granulomatous disease (D). Cyclical thrombocytopenia produces bleeding manifestations rather than the recurrent neutropenic infections and oral ulceration described here (E).
Reference: Fragni M et al. Cyclic Neutropenia Mimicking Crohn's Disease: Two Case Reports and a Narrative Review. Journal of Clinical Medicine. 2023;12:6323. https://pubmed.ncbi.nlm.nih.gov/37834967/