2 The histopathology of chronic myeloproliferative diseases

This chapter discusses the histopathology of five groups of chronic myeloproliferative diseases: chronic myeloid leukaemia, polycythaemia vera, essential thrombocythaemia, chronic idiopathic myelofibrosis and unclassifiable myeloproliferation. Histological staging of the four haematologically defined diseases is performed by grading the three most prominent variables: megakaryocytes, fibres and blasts. Histological outcome is correlated to the staging of diagnostic bone marrow biopsies; megakaryocytic involvement is correlated with the risk of myelofibrosis. An excess of blasts is related to the risk of leukaemic transformation. The progression of myelofibrosis depends on the grade of fibre increase at diagnosis. These three statements are highly significant and valid for all types of chronic myeloproliferative disorders. The results of cytogenetics are discussed in relation to the histological classification for these patient groups. Changes in bone marrow histology following myelosuppressive therapy is presented. Prospective studies under standardized protocol therapy are recommended, so that the long-term effects of therapy can be assessed.