HAEMOSTASIS STUDY IN THALASSAEMIC PATIENTS WITH VARIOUS TYPES OF LIVER DISEASE. 179

Abstract

A pannel of haemostatis parameters (I, II, V, VII, VIII, IX, X, XI, XII, ATIII, PC, PS, Plg, FPA, TAT, PAP, D-dimers) were studied in 4 groups of patients with thalassemis major. Group A: 14 patients ([horizontal bar over]x age 22.5 ± 4.0 yrs) with compensated cirrhosis ([horizontal bar over]x ferritin levels 3.148 ± 2.004 ng/ml), Group B: 16 pts ([horizontal bar over]x age 20.1 ± 3.0 yrs) with chronic active or persistent hepatitis C ([horizontal bar over]x ferritin levels 2.835 ± 1.246 ng/ml), Group C: 15 pts ([horizontal bar over]x age 24.0 ± 5.3 yrs) with haemosiderosis ([horizontal bar over]x ferritin levels 3.189 ± 1.935 ng/ml) and Group D: 18 young pts ([horizontal bar over]x age 7.1 ± 2.1 yrs) without hepatitis ([horizontal bar over]x ferritin levels 2.317 ± 960 ng/ml). All patients were on chelation thcrapy with desferrioxamine. A statistically significant reduction in fibrinogen, FII, FX, Antithrombin III(ATIII), Protein C (PC), Protein S (PS) and Plasminogen (Plg) values was observed in all groups of patients as compared to controls. Increased FVIII and PS levels were found in Group A and D respectively. Elevated Fibrinopeptide A (FPA) levels (a marker of fibrinogen degradation by thrombin) were demonstrated in 40% of pts in Groups A,B and C which were not correlated with indications of coagulation (↑TAT) or fibrinolytic (↑PAP) system activation. Thalassaemic patients are prone to develop liver dysfunction as a result of haemosiderosis or/and HBV or HCV infection, nevertheless the haemostatic defect differed from Group to Group of patients and in general it was mild (probably due to a selective production of the haemostasis components by the hepatocytes) and not associated with haemorrhagic or thrombotic complications, at least during the period of the study.