Thrombophilic genotypes, natural anticoagulants, and plasma homocysteine in myeloproliferative disorders: relationship with splanchnic vein thrombosis and arterial disease.
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 C(677)T, factor V Leiden, prothrombin G(20210)-->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 micromol/L) than NT patients (12.3 +/- 7.7 micromol/L), SVT patients (9 +/- 4.9 micromol/L), and healthy controls (7.9 +/- 3 micromol/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.
Amitrano
L
L
Gastroenterology, A. Cardarelli Hospital, Naples, Italy.
Guardascione
M A
MA
Ames
P R J
PR
Margaglione
M
M
Antinolfi
I
I
Iannaccone
L
L
Annunziata
M
M
Ferrara
F
F
Brancaccio
V
V
Balzano
A
A
D000328:Adult; D000368:Aged; D001157:Arterial Occlusive Diseases / Q000097:blood / Q000453:epidemiology / Q000209:etiology*; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood*; D016022:Case-Control Studies; D015897:Comorbidity; D005260:Female; D005838:Genotype; D006710:Homocysteine / Q000097:blood*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009196:Myeloproliferative Disorders / Q000097:blood* / Q000150:complications; D015995:Prevalence; D012189:Retrospective Studies; D013152:Splanchnic Circulation; D019851:Thrombophilia / Q000097:blood* / Q000235:genetics; D020246:Venous Thrombosis / Q000097:blood / Q000453:epidemiology / Q000209:etiology*
D019774:Blood Coagulation Factor Inhibitors; D006710:Homocysteine