ABC of clinical haematology: Polycythaemia, primary (essential) thrombocythaemia and myelofibrosis

摘要

Primary polycythaemia (polycythaemia rubra vera), primary (or essential) thrombocythaemia, and myelofibrosis are all clonal disorders originating from a single aberrant neoplastic stem cell in the bone marrow. They are generally diseases of middle or older age and have or may develop common features including a small potential for transforming to acute leukaemia. Myelofibrosis may arise de novo or result from progression of primary polycythaemia or primary thrombocythaemia. Treatment of primary polycythaemia and primary thrombocythaemia can greatly influence prognosis, hence the importance of diagnosing these rare disorders. They need to be distinguished from other types of polycythaemia (secondary polycythaemia, apparent polycythaemia) and other causes of a raised platelet count (secondary or reactive thrombocytosis), whose prognosis is different.