Abstract
Acute spontaneous, agnogenic agranulocytosis which occurred in periodic cycles of three weeks is described. Episodes were progressively severe and characterized by fever, stomatitis and gangrenous ulcerations of the skin. Penicillin was used successfully to control the sepsis. The bone marrow showed a depression and maturation arrest of granulopoiesis at the promyelocyte-myelocyte level and disappearance of neutrophiles from the peripheral blood before each attack. Return of granulopoiesis to normal and reappearance of neutrophiles in the peripheral blood prededed recovery. Following palliative splenectomy periods of neutro-penia occurred at long and irregular intervals without toxic symptoms. Higher neutrophile counts were maintained and the bone marrow improved. The spleen showed follicular hyper-plasia.

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