Philadelphia Chromosome-Negative Chronic Myeloproliferative Disease

作者：Thiele Juergen^*

来源：American Journal of Clinical Pathology, 2009, 132(2): 261-280.

DOI：10.1309/AJCPR8GINMBDG9YG

• Chronic eosinophilic leukemia
• Myeloid and lymphoid neoplasms with eosinophilia
• Genetic abnormalities
• Neutrophilic leukemia
• Polycythemia vera
• Primary myelofibrosis
• Essential thrombocythemia
• JAK2(V617F)
• Reactive thrombocytosis
• Reactive myelofibrosis
• Chronic myeloproliferative neoplasms

Session 2 of the 2007 Workshop of the Society for Hematopathology/European Association for Haematopathology was focused on Philadelphia chromosome-negative chronic myeloproliferative diseases (Ph-MPDs), recently termed chronic myeloproliferative neoplasms. The presented and submitted cases highlighted some important issues and also impending problems associated with the diagnosis and classification. Cases included predominantly rare entities like chronic eosinophilic leukemia and related disorders, chronic neutrophilic leukemia, and others with specific genetic abnormalities that allowed molecularly targeted therapy. In this context, the distinctive role of a positive JAK2(V617F) mutation fir the diagnosis of Ph-MPD was underscored, including entities with a low allele burden and the discrimination from reactive disorders (autoimmune myelofibrosis, reactive thrombocytosis). Although novel genetic and molecular approaches have significantly improved the way we classify Ph- MPD, a combined clinicopathologic approach, including representative bone marrow specimens, still remains the yardstick for diagnosis.

• 出版日期2009-8