Abstract
Summary: A granulocytic sarcoma expanded the malar region in a patient with proven myelofibrosis over a 22 month period before undergoing rapid increase in size concomitantly with transformation to acute granulocytic leukaemia in the marrow and the widespread appearance of subcutaneous tumour deposits. Rapid response was obtained with local radiotherapy, and the systemic disease manifestations were controlled on combination courses of oral 4′-demethoxydaunorubicin and the epipodophyllotoxin VP16-213. This appears to be the first example of a granulocytic sarcoma occurring in a patient with myelofibrosis.