Treatment of polycythaemia vera and essential thrombocythaemia.

Abstract

The clinical course in both polycythaemia vera (PV) and essential thrombocythaemia (ET) is characterized by significant thrombohaemorrhagic complications and variable risk of disease transformation into myeloid metaplasia with myelofibrosis or acute myeloid leukaemia. Randomized studies have shown that the risk of thrombosis was significantly reduced in ET with the use of hydroxyurea (HU) and in PV with the use of chlorambucil or 32P. However, the use of chlorambucil or 32P has been associated with an increased risk of leukaemic transformation. Subsequently, other studies have suggested that both HU and pipobroman may be less leukaemogenic and as effective as chlorambucil and 32P for preventing thrombosis in PV. However, the results from these prospective studies have raised concern that even HU and pipobroman may be associated with excess leukaemic events in both ET and PV. The recent introduction of anagrelide as a specific platelet-lowering agent, the demonstration of treatment efficacy with interferon-alpha, and the revived interest in using low-dose acetylsalicylic acid provide the opportunity to initiate prospective randomized studies incorporating these treatments.

• Authors: A Tefferi, M N Silverstein
• Journal: Bailliere's clinical haematology (Baillieres Clin Haematol) Vol. 11 Issue 4 Pg. 769-85 (Dec 1998) ISSN: 0950-3536 [Print] England
• PMID: 10640216 (Publication Type: Journal Article, Review)

Chemical References

• Interferon-alpha
• Phosphorus Radioisotopes
• Quinazolines
• Chlorambucil
• Pipobroman
• anagrelide
• Aspirin
• Hydroxyurea

Topics

• Adult
• Aged
• Aspirin (therapeutic use)
• Chlorambucil (adverse effects, pharmacology)
• Disease Progression
• Female
• Hemorrhage (epidemiology, etiology, prevention & control)
• Humans
• Hydroxyurea (adverse effects, therapeutic use)
• Interferon-alpha (therapeutic use)
• Leukemia, Myeloid (etiology, prevention & control)
• Leukemia, Radiation-Induced (etiology)
• Male
• Middle Aged
• Phlebotomy
• Phosphorus Radioisotopes (adverse effects, therapeutic use)
• Pipobroman (adverse effects, therapeutic use)
• Polycythemia Vera (complications, drug therapy, radiotherapy, therapy)
• Primary Myelofibrosis (etiology, prevention & control)
• Prospective Studies
• Quinazolines (therapeutic use)
• Thrombocythemia, Essential (drug therapy, radiotherapy, therapy)
• Thrombosis (epidemiology, etiology, prevention & control)