Thrombophilic genotypes, natural anticoagulants, and plasma homocysteine in myeloproliferative disorders: Relationship with splanchnic vein thrombosis and arterial disease

• Published in: American journal of hematology 2003-02, Vol.72 (2), p.75-81
• Main Authors: Amitrano, L., Guardascione, M.A., Ames, P.R.J., Margaglione, M., Antinolfi, I., Iannaccone, L., Annunziata, M., Ferrara, F., Brancaccio, V., Balzano, A.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Arterial Occlusive Diseases - blood; Arterial Occlusive Diseases - epidemiology; Arterial Occlusive Diseases - etiology; Biological and medical sciences; Blood Coagulation Factor Inhibitors - blood; Case-Control Studies; Comorbidity; Female; Genotype; Hematologic and hematopoietic diseases; homocysteine; Homocysteine - blood; Humans; Male; Medical sciences; Middle Aged; Multivariate Analysis; myeloproliferative disorders; Myeloproliferative Disorders - blood; Myeloproliferative Disorders - complications; Platelet diseases and coagulopathies; Prevalence; protein C; Retrospective Studies; Splanchnic Circulation; Thrombophilia - blood; Thrombophilia - genetics; thrombosis; Venous Thrombosis - blood; Venous Thrombosis - epidemiology; Venous Thrombosis - etiology
• ISSN: 0361-8609; 1096-8652
• DOI: 10.1002/ajh.10254

The contribution of pro‐thrombotic factors towards the development of arterial disease (AD) and splanchnic vein thrombosis (SVT) was retrospectively evaluated in 79 patients (39M, 40F, mean age 55 ± 16 years) with myeloproliferative disorders (MPD) (essential thrombocythemia [n = 26], primary proliferative polycythemia [n = 27], and idiopathic myelofibrosis [n = 26]). Of these, 18 had AD and 17 SVT, the remaining 44 were non‐thrombotic (NT). Plasma concentrations of natural anticoagulants, plasma homocysteine (HC), IgG anticardiolipin antibodies (aCL), and thrombophilic genotypes (methylenetetrahydrofolate reductase C677T, factor V Leiden, prothrombin G20210→A) were determined. Isolated protein C deficiency was found in 23% of patients from the SVT group, in 5% from the AD group, in 6.8% from the NT group, and in 1% of historical controls (P = 0.0001). The prevalence of thrombophilic genotypes and that of the other natural anticoagulants did not differ across the groups. The proportion of patients with elevated plasma HC was 66% in the AD group, 27% in the non‐thrombotic group, 12% in the SVT group and 4.5% in the control group (P < 0.0001). Patients with AD had higher plasma HC (24.4 ± 23 μmol/L) than NT patients (12.3 ± 7.7 μmol/L), SVT patients (9 ± 4.9 μmol/L), and healthy controls (7.9 ± 3 μmol/L) (P < 0.0001). In a logistic regression model lower protein C was independently associated with SVT, whereas elevated plasma HC was independently associated with AD. Measurement of plasma HC and protein C in MPD may identify patients more likely to suffer arterial disease and splanchnic vein thrombosis and who may require plasma HC lowering in the former case. Am. J. Hematol. 72:75–81, 2003. © 2003 Wiley‐Liss, Inc.