We conducted a multicenter prospective study for evaluating the utility and prognostic markers of recombinant human soluble thrombomodulin (rTM) treatment for acute disseminated intravascular coagulation (DIC) by various types of hematologic malignancies. The study comprised 30 patients with DIC due to hematologic diseases without severe infection. DIC improved in 15 patients and 20 were alive on day 28. Univariate analyses showed that, in comparison with patients who had survived on day 28, patients who had not survived on day 28 showed significantly higher plasma levels of plasminogen activator inhibitor-I (PAI-I) and significantly lower plasma activity of a disintegrin and metalloproteinase with a thrombospondin Type 1 motif, member 13 (ADAMTS-13). Moreover, multivariate logistic regression analysis identified a significant association between plasma ADAMTS-13 activity before treatment and survival on day 28 (P= 0.034). In particular, patients with lower ADAMTS-13 activity (≤65%) had a poorer survival rate than those with a higher activity (P= 0.042). These findings suggest that the plasma ADAMTS-13 activity at the time of DIC diagnosis might help to predict the prognosis of patients treated with rTM for DIC associated with hematologic malignancies.