The limited effects of current treatments of primary myelofibrosis (PM) led us to prospectively evaluate recombinant interferon-α (rIFNα) in "early" PM patients with residual hematopoiesis and only grade 1 or 2 myelofibrosis. Seventeen patients meeting World Health Organization PM diagnostic criteria received either rIFNα-2b 500 000 to 3 million units 3 times weekly, or pegylated rIFNα-2a 45 or...

OBJECTIVE Histopathology of bone marrow (BM) biopsies plays a crucial role in the interdisciplinary approach to diagnosis and classification of Ph-negative chronic myeloproliferative disorders. Based on careful clinicopathologic studies, BM features are critical determinants that help to predict overall prognosis, to detect complications such as progression to myelofibrosis and blast crisis, an...

Several international working groups cooperated to propose new diagnostic guidelines for polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) to the steering committee of the World Health Organization. Because JAK2 mutation status presents a decisive diagnostic test in PV, this feature was introduced as a major criterion. Minor criteria, such as characteristic...

Introduction Myelofibrosis with myeloid metaplasia (MMM) represents both agnogenic myeloid metaplasia (AMM) and the fibrotic stages of polycythemia vera (PV) and essential thrombocythemia (ET). The latter two conditions are also referred to as post polycythemic myelofibrosis (PPM) and post thrombocythemic myelofibrosis (PTM). MMM is currently classified under the broad category of chronic myelo...

BACKGROUND Myelofibrosis and acquired hemophilia A is a rare association. To the best of our knowledge only one case of myelofibrosis and acquired hemophilia A has been previously described. CASE PRESENTATION A 66-year-old Caucasian man diagnosed with myelofibrosis evolving in acute myeloid leukemia was referred to us for postoperative bleeding. Hemostatic studies showed prolonged activated p...

We investigated 15,542 patients with suspected BCR-ABL1- negative myeloproliferative or myelodysplastic/myeloproliferative neoplasm (including 359 chronic myelomonocytic leukemia) by a molecular marker set. JAK2V617F was detected in the suspected categories as follows: polycythemia vera 88.3%, primary myelofibrosis 53.8%, essential thrombocythemia 50.2%, and not further classifiable myeloprolif...

Primary myelofibrosis (PMF) is a fatal neoplastic disease characterized by clonal myeloproliferation and progressive bone marrow (BM) fibrosis thought to be induced by mesenchymal stromal cells stimulated by overproduced growth factors. However, tissue fibrosis in other diseases is associated with monocyte-derived fibrocytes. Therefore, we sought to determine whether fibrocytes play a role in t...

The Wilms tumor gene WT1 is a useful marker of clonal hematopoiesis and it has been shown to be a good marker of residual disease and it reflects the response to therapy. Although myelofibrosis is characterized by mutations of JAK2 and calreticulin (CALR), these mutations are not useful to monitor response to therapy. In this study we demonstrated that in patients affected by myelofibrosis WT1 ...

Idiopathic myelofibrosis is a rare chronic myeloproliferative disease leading to extramedullary hematopoiesis (myeloid metaplasia) with splenomegaly. The liver and less frequently other organs including the lung can be involved, therefore portal hypertension is relatively common. Pulmonary hypertension (PH) is only occasionally reported, although recent studies have suggested an association bet...

A 28-year-old man with Philadelphia chromosome positive chronic granulocytic leukaemia developed extensive bone marrow and bone infarction which was associated with anaemia and thrombocytopenia. He survived 20 months from the first symptoms of bone marrow infarction; during this time he developed myelofibrosis and osteosclerosis followed by blastic transformation. Extensive bone marrow infarcti...