Other Acquired Disorders of Hemostasis As discussed above, acquired disorders of hemostasis include liver disease, vitamin K deficiency, and development of inhibitors to VWF or other coagulation factor proteins, often in the setting of an autoimmune condition Patients with chronic liver disease and vitamin K deficiency may benefit from vitamin K supplementation When patients have bleeding, replacement of factor deficiencies and platelets, if low, may be useful Patients with renal failure require optimal management of their uremia and may benefit from platelet transfusion in the right clinical setting For patients who develop inhibitors to coagulation factors (acquired hemophilia), management is complex and should involve a hematologist Clinical Indications for Discharge or Admission Patients with uncontrolled bleeding or with clinical concern for bleeding risk should be admitted for ongoing inpatient management and diagnostic evaluation if necessary If a bleeding disorder is suspected on the basis of initial laboratories or patient or family history, but the child is not actively bleeding, additional evaluation may occur in the outpatient setting with a hematology consultation HEMOPHILIA Goals of Treatment Early identification of bleeding in hemophilia patients is crucial for treating lifethreatening bleeds and preventing the long-term sequelae of recurrent joint bleeding CLINICAL PEARLS AND PITFALLS Drugs that interfere with platelet function (e.g., aspirin, NSAIDs) should not be given to patients with hemophilia Emicizumab, used for prophylaxis in hemophilia A, interferes with standard coagulation assays, artificially shortening the aPTT and increasing FVIII activity levels Current Evidence Hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) are Xlinked disorders of hemostasis Hemophilia A accounts for roughly 85% of cases (incidence ∼1:5,000 males), and hemophilia B for the remaining 15% (incidence ∼1:30,000 males) The bleeding phenotype can usually be predicted from the level of factor coagulant activity If factor activity is less than 1% (severe hemophilia), bleeding episodes can occur spontaneously Patients with moderate hemophilia (factor level between 1% and