Volumen: 17 # Number : 2
Publication Date : Mayo - Agosto Year: 2013
Authors: Musso, AM
Abstract: PV is an acquired clonal, chronic, progressive disease. Two phases can be recognized, the erythrocytotic phase (EP) and the spent phase (PPMM). The main complications of PV are vascular
thrombosis, bleeding, myelofibrosis, myelodysplasia and acute leukemia.
The prognosis of a patient with PV depends on the nature and severity
of the complications; the duration of the EP previous to the development of PPMM, myelodysplasia or acute leukemia; and the treatment instituted during the EP phase of the disease.
The EP has different degrees of severity. Appropriate treatment should be given to the patient in the early stage of the disease, before
serious complications develop.
Patients at low-risk for thrombosis or bleeding, with an indolent form of PV can be treated with phlebotomy plus low-dose aspirin (100 mg/day), to maintain hematocrit <45%.
Patients at high risk of thrombosis and/or bleeding, with progressive
leukocytosis or thrombocytosis, enlargement of the spleen, uncontrolled symptoms, or intolerance to or high need of phlebotomy,
should receive cytoreductive therapy.
Treatment with phlebotomy, hydroxyurea and pegylated interferon-
alfa from the beginning, plus low-dose aspirin, can be an appropriate cytoreductive therapy. Hydroxyurea given for a short period of time, until interferon is effective, has a lower risk of adverse
events.
Pegylated interferon-alfa therapy has neither oncogenic nor teratogenic effects, is associated with low risk of thrombosis, can be given to pregnant women and can be used at any age.
It is possible that pegylated interferon-alfa combined with Ruxolitinib
will be of help in the treatment of patients with PPMM in the future. Allogeneic stem cell transplantation for PPMM though effective in some cases has significant morbimortality.
Key words: Policytemya Vera, treatment
Pages : 147-152
|
