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Циклічна нейтропенія

ORPHA:2686· ICD-10 D70· Cyclic neutropenia

Визначення(English summary)

A rare primary immunodeficiency characterized by regular oscillations in blood neutrophil counts from normal or subnormal levels to severe neutropenia, usually with a cycle length of about 21 days. Symptoms during the neutropenic phase include fever, mouth ulcers, but also pneumonia, and peritonitis, among others. Mode of inheritance is autosomal dominant.

Поширеність
1-9 / 1 000 000
Успадкування
Autosomal dominant
Вік початку
All ages