Abstract
Myeloproliferative neoplasms (MPNs) are clonal stem cell disorders characterized by the presence of JAK2V617F mutation. Thrombohemorrhagic as well as autoimmune or inflammatory phenomena are common clinical outcomes of these disorders. Recent studies have shown that abnormality in frequency and function of blood cells manifested by an alteration in CD markers’ expression patterns play a key role in these complications. So, there may be a relationship between CD markers’ expressions and prognosis of JAK2V617F positive MPNs. Therefore, in this review, we have focused on these abnormalities from the perspective of changing expressions of CD markers and assessment of the relationship between these changes with prognosis of JAK2V617F positive MPNs. It can be stated that the abnormal expression of a large number of CD markers can be used as a prognostic biomarker for clinical outcomes including thrombohememorrhagic events, as well as autoimmune and leukemic transformation in JAK2V617F positive MPNs. Considering the possible role of CD markers’ expressions in JAK2V617F MPNs prognosis, further studies are needed to confirm the relationship between the expression of CD markers with prognosis to be able to find an appropriate therapeutic approach via targeting CD markers.
Highlights
Janus activating kinase 2 (JAK2) V617F is a common mutation in Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), which include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF).[1]
Several studies have indicated that the annual incidence of thrombotic complications in MPNs is around 1-10% and that there is a correlation between MPN manifestations and JAK2V617F mutant alleles burden.[12-14]
The JAK2V617F mutation can affect the circulating blood cell counts, this mutation does not seem to be responsible for all clinical findings in these disorders by itself; a range of factors such as variation in the number and function of different blood cells, increased levels of circulating microparticles, leuko-platelet microaggregates, and altered function of endothelial cells may contribute to the occurrence of these complications.[9,17,18]
Summary
Janus activating kinase 2 (JAK2) V617F is a common mutation in Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), which include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF).[1]. The presence of excess circulating endothelial cells, which is detected by the expression of MDVs markers, may be a poor prognostic factor for increasing incidence of angiogenesis and the resulting thrombotic events in these disorders It seems that the flow cytometric immunophenotyping of expression levels of these markers from the viewpoint of clinical outcomes and response to treatment in JAK2V617F positive MPNs can provide useful information on the prognosis of these disorders. It has been shown that patients with a higher expression of CD177 are at increased risk of thrombotic and bleeding complications due to increased circulating neutrophils,[105] it is inferred that the overexpression of this marker in JAK2V617F positive MPNs could be a poor prognostic factor for the presence of bacterial contamination in these disorders. Subsequent timely therapeutic interventions by an appropriate approach may minimize the unfavorable clinical outcomes of leukemic transformation
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