Rebalanced hemostasis in patients with idiopathic thrombocytopenic purpura

Abstract

Previous laboratory and clinical data have shown evidence for the concept of rebalanced hemostasis in liver disease. We evaluate whether this concept of rebalanced hemostasis can be applied in patients with idiopathic thrombocytopenic purpura (ITP). Twenty patients with ITP (platelet count < 100 × 109 /l) who visited our hospital were enrolled. We measured the von Willebrand factor (vWF) antigen levels and performed native blood thromboelastography (TEG) to evaluate the hemostasis. As a subgroup analysis, we compared patients with elevated vWF levels with those with normal levels. Bleeding symptoms of the patients were followed up for 6 months. The mean (SD [IQR]) platelet count was 44.23 × 109 /l (25.78 [27.00–60.50]). The following TEG parameters were within the normal range in most patients (number of patients with a normal value): clotting time (17), clot formation time (17), α-angle (15), maximum clot formation (10), and maximum lysis (12). The mean (SD) vWF antigen level (%) was 163% (80). There were eight patients (40%) with elevated vWF antigen levels [218% (104) vs. 126% (19), p = 0.007, elevated vs. normal patients, respectively]. Those with elevated vWF antigen levels were older [58 year (10) vs. 40 year (13), p = 0.004] and had a longer disease status [67 months (39) vs. 33 months (25), p = 0.028]. Although the platelet count was not different, the CFT was shorter [287 (104) vs. 561 (291), p = 0.042] and the α-angle was larger [49 (6) vs. 34 (15), p = 0.033] in those with elevated vWF antigen levels. There were no patients with major bleeding events during the follow-up period. Four patients showed minor bleeding events (n = 1 vs. n = 3, elevated vs. normal patients, respectively). We found that the vWF antigen level was elevated and the TEG profiles were better in older ITP patients with longer disease statuses. Patients with ITP appeared to achieve a rebalance hemostasis through an elevation of their plasma vWF antigen levels and hemostatic changes that promote thrombosis. Measuring the vWF antigen levels and performing TEG analysis can help determine the treatment strategy in ITP patients.