title: High frequency of endothelial colony forming cells marks a non-active myeloproliferative neoplasm with high risk of splanchnic vein thrombosis.
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 7.8×10(9)/L or lower, and platelet count of 400×10(9)/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.
authors.affiliation: Unit of Clinical Epidemiology and Center for the Study of Myelofibrosis, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
authors.forename: Vittorio
authors.initials: V
authors.lastname: Rosti
authors.forename: Elisa
authors.initials: E
authors.lastname: Bonetti
authors.forename: Gaetano
authors.initials: G
authors.lastname: Bergamaschi
authors.forename: Rita
authors.initials: R
authors.lastname: Campanelli
authors.forename: Paola
authors.initials: P
authors.lastname: Guglielmelli
authors.forename: Marcello
authors.initials: M
authors.lastname: Maestri
authors.forename: Umberto
authors.initials: U
authors.lastname: Magrini
authors.forename: Margherita
authors.initials: M
authors.lastname: Massa
authors.forename: Carmine
authors.initials: C
authors.lastname: Tinelli
authors.forename: Gianluca
authors.initials: G
authors.lastname: Viarengo
authors.forename: Laura
authors.initials: L
authors.lastname: Villani
authors.forename: Massimo
authors.initials: M
authors.lastname: Primignani
authors.forename: Alessandro M
authors.initials: AM
authors.lastname: Vannucchi
authors.forename: Francesco
authors.initials: F
authors.lastname: Frassoni
authors.forename: Giovanni
authors.initials: G
authors.lastname: Barosi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D042783:Endothelial Cells / Q000166:cytology*; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D016013:Likelihood Functions; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000473:pathology*; D016017:Odds Ratio; D011087:Polycythemia Vera / Q000473:pathology; D012680:Sensitivity and Specificity; D013152:Splanchnic Circulation; D013921:Thrombocytopenia / Q000473:pathology; D020246:Venous Thrombosis / Q000473:pathology*
chemical_list: D006454:Hemoglobins