A Case of Cyclic Agranulocytosis

Summary After a brief reference to three previously reported cases of cyclic neutropenia without any demonstrable cause, an account is submitted of a similar case, a boy aged 16 years who from the age of 14 months has had attacks a t intervals of three weeks with ulcerations in the mouth, rise of temperature, and general malaise, but with milder symptoms the last two years. During nine months' observation these attacks coincided with a neutropenia in the blood and, in the bone marrow, with an arrest in the development of the iieutrophiles at the myelocyte‐promyelocytic stage. In the remission intervals a mild leucopenia and low neutrophile count persisted on solitary occasions. Titrations of the urine for hormones showed an occasional folliculin and prolan value that bordered on the pathologic. A constant finding was low serum iron, which rose to normal during iron‐treatment but immedeately fell again o n withdrawal of this medication. With reference t o the cause of the pathologic picture a cyclic infection or a primary disturbance of the endocrinous and central nervous regulation of the blood has been discussed. It is pointed out that the neutropenia also followed an approximately three‐weeks' cycle in previously reported cases, and that the condition may be a disease sui generis that is commoner than is thought. Attention is drawn t o its resemblance to cases described as periadenitis mucosa necrotica and ulcus neuroticum oris. No effect on the morbid state was obtained from reiterated smallpox‐vaccinations, pentnucleotide injections, administration of liver extracts and iron, and a plentifull supply of vitamins B and C.