Further Outpatient Care
In the plethoric phase, patients initially require close follow-up for monitoring of blood counts. The dose of hydroxyurea must be closely monitored until a steady state is achieved and phlebotomy may occasionally be required in symptomatic patients.
In the spent phase, therapy needs to switch from removal of cells to transfusion of cells to relieve anemia. Leukemic transformation needs to be managed expectantly but has a poor prognosis.
Further Inpatient Care
Most patients with polycythemia vera (PV) can be managed as outpatients.
Occasionally a symptomatic patient who has an extremely high hematocrit may need hospitalization for emergent phlebotomy. These patients should receive aggressive volume replacement with saline. Their CBC counts should be closely monitored.
Complications
As outlined in detail above, potential complications of this disorder are primarily thromboembolic and hematologic.
Increased cardiovascular morbidity and mortality can be significant, although appropriate treatment is felt to significantly reduce these risks. Unfortunately, whether current therapies can reduce the risk of hematologic transformation is unclear. Therapies that reduce the mutant clone population, such as interferon and possibly JAK2 inhibitors in the future, will hopefully decrease this risk.
Prognosis
For many patients, a normal or near normal life span can be anticipated. However, polycythemia vera carries significant potential morbidity and mortality, even when correctly treated.
As outlined by Marchioli et al, cardiovascular events are more common in this population and pose an ever present risk for these patients. [14]
Transformation to myelofibrosis decreases anticipated survival and, although uncommon, transformation to acute leukemia portends a very poor prognosis.
Please refer to Morbidity/Mortality for further details.
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Bone marrow film at 400X magnification demonstrating dominance of erythropoiesis. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
