Homeostatic disorders

462 Cyclic neutropenia: Rare. Neutropenia arising every 21d lasting 3-6d. Counts may fall <0.2 x 109/L. Associated with episodes of fever, malaise, mucous membrane ulcers, lymphadenopathy. Serious infection can arise and deaths May improve after puberty. Usually positive family history when problem encountered in childhood. Treatment supportive with antibiotics. G-CSF may be useful.

Chronic benign neutropenia: More common. Persistent rather than cyclical, though often variable without clear periodicity. Neutrophil count usually >0.5 x 109/L, so clinically mild or silent. May have autosomal dominant or autosomal recessive family history. Variable severity, often mild with few problems. To be distinguished from severe congenital neutropenia (ffl Kostmann's syndrome, p459) by milder clinical course and (usually) later presentation. Therapy not usually necessary.

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