Objective To analyze the pathogenesis,therapy and outcome of pediatric cases with coagulation disorders (CD).Methods All these 137 patients were diagnosed as CD with the methods of hemoglutination five items and/or disseminated intravascular coagulation indexes.Then activity of specific coagulation factors,morphology of bone marrow,hepatorenal function and some other relative tests were performed to find out the cause of CD or the primary disease.Results Forty-three cases were diagnosed as genetic CD with 29 as hemophilia A,4 as hemophilia B and 10 as Von Willebrand disease;while the other 94 patients as acquired CD with 15 as vitamin K-dependence coagulation factor deficiency,22 as hepatic dysfunction,30 as disseminated intravascular coagulation and 1 as thrombotic thrombocytopenic purpura.Genetic CD was treated with replacement therapy to reduce the complication.There was 1 case in this group died of intracranial hemorrhage.Acquired CD was treated with short-term,specific and necessary replacement therapy on the basis of reasonable treatment of primary diseases.Eleven cases died finally in this cohort with 7 cases as liver failure and the other 4 cases as terminal leukemia or lymphoma.Conclusion Pediatric patients with CD were caused by genetic or acquired diseases.In clinic the reason of CD was mainly acquired.The treatment of genetic CD is the replacement of specific coagulation factor for life-long term.The outcome dependes on the lack of degree.While the therapy for acquired CD aims at the primary disease.The principle of blood transfusion is short-term and the outcome dependes on the therapic effects of primary diseases.