Haemostatic abnormalities, cardiac involvement and serum tumor necrosis factor levels in X-linked dystrophic patients

Left ventricular thrombosis and systemic emboli have been demonstrated to c omplicate cardiomyopathy in Duchenne and Becker muscular dystrophy (DMD, BM D). We investigated plasma levels of prothrombin fragment 1+2 (F1+2), throm bin-antithrombin III complex (TAT) and circulating levels of tumor necrosis factor-alpha (TNF-alpha), a procoagulant cytokine that has been shown to b e elevated in patients with depressed cardiac function, in 20 patients with DMD and 12 patients with BMD as compared with 30 age-matched control subje cts. Significantly elevated levels of F1+2 (DMD: 1.4 +/- 0.8 nmol/l; BMD: 1 .8 +/- 0.8 nmol/l vs, controls: 0.7 +/- 0.2 nmol/l, p <0.01 and p <0.001, r espectively), TAT complexes (DMD: 4.7 +/- 2.7 mu g/l, BMD: 5 +/- 2.3 mu g/l vs. controls: 1.6 +/- 0.5 mu g/l, p <0.001) and TNF-alpha (54 +/- 9 vs. 25 +/- 7 pg/ml, p <0.001) were observed in patients with the dystrophic disea se compared to control subjects. A significantly negative correlation was a lso found between F1+2 and TAT complexes and left ventricular ejection frac tion (r = -0.65, p <0.0001; r = -0.80, p <0.0001, respectively) and a posit ive correlation between F1+2 and TAT complexes and serum TNF-alpha levels ( r = 0.67, p <0.0001; r = 0.70, p <0.0001, respectively). Our results indica te a hypercoagulable state in X-linked dystrophic patients. A possible rela tionship between haemostatic activation, left ventricular dysfunction and T NF-alpha system upregulation may be suggested.