Survival in symptomatic patients without treatment is estimated to be between 6 and 18 months, and with appropriate therapy >10 years. However, the mean survival is influenced by the age at diagnosis. Thus, younger patients diagnosed and managed appropriately can have longer survivals. The main causes of death are thrombosis (29%), hematologic malignancies in those treated in previous eras with alkylating agents (23%), nonhematologic malignancies (16%), hemorrhage (7%), and myelofibrosis with myeloid metaplasia (3%).6

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