Primary myelofibrosis (PMF) is a stem cell-derived clonal myeloproliferative disorder (MPD) that is characterized clinically by anemia, marked enlargement of the spleen and liver, and severe constitutional symptoms. Peripheral blood findings include the presence of immature myeloid cells including nucleated red blood cells, immature granulocytes, and teardrop-shaped erythrocytes. The bone marrow histology exhibits reticulin and collagen fibrosis, osteosclerosis, and angiogenesis.
The blood and bone marrow features associated with PMF are discovered either de novo (i.e., PMF) or in the setting of either polycythemia vera (post-PV MF) or essential thrombocythemia (post-ET MF). PMF is also known by many synonyms. However, the use of the term “PMF” was recently endorsed by the International Working Group for...