| 1634 | haematologica | 2009; 94(12) The World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues includes within myeloid neoplasms the category “Myelodysplastic/myeloproliferative neoplasms”. According to Vardiman et al., these are “clonal myeloid neoplasms that at the time of initial presentation have some clinical, laboratory or morphologic findings tha...

A secondary myeloid or lymphoid neoplasm is not infrequently associated with a primary tumor, solid or hematopoietic, post cytotoxic treatment or radiation. Concurrent secondary neoplasms derived from distinct myeloid and lymphoid cell origins are rare. It is not only a diagnostic challenge, but makes for difficult treatment management. We report a very rare occurrence in 63-year-old male with ...

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm (MPN) which characterized by proliferation of red blood cells, white cells and platelets. PV associated with the JAK2 V617F mutation. The presenting symptom variable. Headache, weakness, fatigue, pruritus, dyspepsia, dizziness, visual disturbances may be seen in patients. Severe complications such as stroke, acute myocardial infarc...

Our understanding of inflammation's role in the pathogenesis of myeloproliferative neoplasm (MPN) is evolving. The impact of chronic inflammation, a characteristic feature of MPN, likely goes far beyond its role as a driver of constitutional symptoms. An inflammatory response to the neoplastic clone may be responsible for some pathologic aspects of MPN. Moreover, JAK2V617F mutated hematopoietic...

In the diagnostic criteria of these neoplasms, besides haematological tests and osteomedular biopsy an important role is played by the JAK2V617F, CALR and Mpl mutational status testing.1 Numerous researches involved the presence of JAK mutation in increasing the risk of thrombosis; in this way, along with advanced age and history of thrombosis in the classification criteria in “high risk” forms...

JAK2V617F(+) myeloproliferative neoplasms (MPNs) frequently progress into leukemias, but the factors driving this process are not understood. Here, we find excess Hedgehog (HH) ligand secretion and loss of PTCH2 in myeloproliferative disease, which drives canonical and noncanonical HH-signaling. Interestingly, Ptch2(-/-) mice mimic dual pathway activation and develop a MPN-phenotype with leukoc...

We report a 63-year-old woman with myeloproliferative neoplasm (MPN), who presented with inflammatory involvement of the skin. The white cell count was 31,800/μL and the platelet count was 709×10/μL. The positivity of neutrophil alkaline phosphatase was 100% and the score was 281. The bone marrow was hypercellular with enlarged megakaryocytes with mature cytoplasm and multilobulated nuclei (Pic...

The excess release of mediators can cause clinical features such as pruritus, flushing, nausea, vomiting, diarrhoea, abdominal pain, vascular instability and anaphylaxis. Also, complications may arise when mast cells accumulate in the skin, gastrointestinal tract, bone marrow, liver, spleen, and lymph nodes. [2] The clinical features of systemic mastocytosis are caused by accumulation of clonal...

CONTEXT Alterations in megakaryocyte morphology are the hallmark of myeloproliferative neoplasms (MPNs). These neoplasm are also associated with Janus kinase 2 (JAK2) V617F mutation in nearly 95% patients with polycythemia vera (PV), 40% patients of essential thrombocythemia (ET) and 50% patients of myelofibrosis (MF). The utility of megakaryocyte morphology in these disorders in correlation wi...

Myeloproliferative neoplasms (essential thrombocythemia, ET; polycythemia vera, PV; myelofibrosis, MF) are monoclonal malignancies associated with genomic instability, dysregulated signaling pathways, and subsequent overproduction of inflammatory markers. Acknowledged for their debilitating symptom profiles, recent investigations have aimed to determine the identity of these markers, the upstre...