Abstract
Increased mobilization of circulating endothelial progenitor cells may represent a new biological hallmark of myeloproliferative neoplasms. We measured circulating endothelial colony forming cells (ECFCs) in 106 patients with primary myelofibrosis, fibrotic stage, 49 with prefibrotic myelofibrosis, 59 with essential thrombocythemia or polycythemia vera, and 43 normal controls. Levels of ECFC frequency for patient's characteristics were estimated by using logistic regression in univariate and multivariate setting. The sensitivity, specificity, likelihood ratios, and positive predictive value of increased ECFC frequency were calculated for the significantly associated characteristics. Increased frequency of ECFCs resulted independently associated with history of splanchnic vein thrombosis (adjusted odds ratio = 6.61, 95% CI = 2.54–17.16), and a summary measure of non-active disease, i.e. hemoglobin of 13.8 g/dL or lower, white blood cells count of 7.8×109/L or lower, and platelet count of 400×109/L or lower (adjusted odds ratio = 4.43, 95% CI = 1.45–13.49) Thirteen patients with splanchnic vein thrombosis non associated with myeloproliferative neoplasms were recruited as controls. We excluded a causal role of splanchnic vein thrombosis in ECFCs increase, since no control had elevated ECFCs. We concluded that increased frequency of ECFCs represents the biological hallmark of a non-active myeloproliferative neoplasm with high risk of splanchnic vein thrombosis. The recognition of this disease category copes with the phenotypic mimicry of myeloproliferative neoplasms. Due to inherent performance limitations of ECFCs assay, there is an urgent need to arrive to an acceptable standardization of ECFC assessment.
Highlights
The classical Philadelphia negative (Ph-neg) myeloproliferative neoplasms, i.e. polycythemia vera, essential thrombocythemia, and primary myelofibrosis, consist of clonal malignancies characterized by the proliferation of one or more myeloid lineages that originate at the level of stem cells
In the cross sectional study, we explored the potential of using patients characteristics to identify the association between patients with increased endothelial colony forming cells (ECFCs) frequency and age, sex, disease duration, hemoglobin, white blood cell count, platelet count, number of circulating CD34+ hematopoietic progenitor cells, serum lactate dehydrogenase value, V617F mutational status of JAK2, diagnostic category and prognostic score (International Working GroupMyelofibrosis Research and Treatment, IWG-MRT [24]), at the time of examination
Our analysis of a large cohort of patients with Ph-neg myeloproliferative neoplasms shows that mobilization of endothelial progenitor cells is consistently higher in patients who received a diagnosis of prefibrotic myelofibrosis
Summary
The classical Philadelphia negative (Ph-neg) myeloproliferative neoplasms, i.e. polycythemia vera, essential thrombocythemia, and primary myelofibrosis, consist of clonal malignancies characterized by the proliferation of one or more myeloid lineages that originate at the level of stem cells. The difficulties in dissecting the continuum of clinical phenotypes are reflected in the recently revised WHO classification [3], which arranges hematological, morphological and molecular parameters to separate the three clinical disorders. Even though this classification has endorsed the concept of ‘‘prefibrotic-early stage of primary myelofibrosis’’, and ‘‘pre-polycythemic phase’’ of polycythemia vera to capture the stepwise evolution of the diseases, the overlapping of clinical phenotypes persists, and the category of ‘‘unclassifiable myeloproliferative neoplasms’’ continues to challenge the system [3]. Colony forming unit-endothelial or CD34-, CD133-, vascular endothelial growth factor receptor 2-
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
