Abstract
The classical myeloproliferative disorders (MPDs) are comprised of the clonal, BCR-ABL-negative, chronic myelold disorders of essential thrombocythemia, polycythemia vera, and myelofibrosis with myeloid metaplasia. Managment of these disorders remains a significant challenge due to the varied range of prognosis and phenotypic manifestations. Curative therapy, achieved in some patients through allogeneic stem cell transplantation, is elusive or inappropriate in most. Additionally, no available medical therapy has been shown to clearly improve survival or delay disease progression. Current management involves an emphasis on prevention of thrombohemorrhagic complications (through aspirin treatment, phlebotomy and cytoreduction in high-risk patients) in early-stage patients and symptomatic care in those with advanced disease. Leukemic transformation from MPDs remains a rapidly fatal complication, unresponsive to current therapies. Recent elucidation of the role of the activating tyrosine kinase mutation JAK2V617F is anticipated to usher in an era of greater understanding and targeted therapy for the MPDs.
Original language | English (US) |
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Pages (from-to) | 515-524 |
Number of pages | 10 |
Journal | Future Oncology |
Volume | 2 |
Issue number | 4 |
DOIs | |
State | Published - Aug 2006 |
Keywords
- Essential thrombocythemia
- MPD therapy
- Myelofibrosis
- Myeloproliferative disorders
- Polycythemia vera
ASJC Scopus subject areas
- Oncology
- Cancer Research