Coagulopathies – Jason Jones

  • If you have a pt with pathological bleeding, first determine platelet vs coagulation disorder
  • Platelet Disorders: Skin/Mucous membrane bleeding; Petechiae; Often mild bleeding following surgery and tends to immediately follow surgery
  • Coagulation defect: Bleeding deep in soft tissues (muscles/joints); hemarthroses, hematomas; Tend to have delayed bleeding after surgery that can be severe
    • Coagulopathies can be divided into Hereditary vs Acquired causes



Hemophilia A (Factor VIII Deficiency) and Hemophilia B (Factor IX Deficiency)

  • Inherited in X-linked Recessive pattern
  • Diagnosis: Isolated prolonged PTT with normalization upon mixing study
  • Management: purified/recombinant Factor VIII or IX. Desmopressin for mild disease
  • If you have patient with hemophilia who needs a procedure/surgery or is acutely ill, this requires consult to benign hematology

von Willebrand Disease (vWD) 

  • Can be hereditary (common) or acquired
  • Abnormal quantity or function of von Willebrand Factor (vWF) needed for platelet function
  • Type 1 (most common): Quantitative defect. Low quantity, normal function of vWF
  • Type 2: Qualitative defect. Normal quantity, abnormal function of vWF. In Type 2A, large multimer formation is impaired
  • Type 3 (rarest): Complete absence of vWF phenotypically similar to hemophilia A
  • Order in Epic: vW Profile = vWF Ag, Factor VIII Activity, Ristocetin Cofactor Activity
  • Management: Desmopressin (DDAVP) can be useful as prophylaxis or treatment





Coagulation Factor Inhibitors

  • Associated with autoimmune disease (paraneoplastic vs. autoantibody)
  • Seen in hemophilia due to frequent treatments with recombinant factors (alloantibody)
  • Diagnosis: Elevated PTT that does not normalize with mixing study
  • Management: Immunosuppression with steroids; cyclophosphamide +/- rituximab
    • Consult Hematology early (rare disorder with major bleeding complications)


Vitamin K Deficiency

  • Caused by malnutrition, liver disease, or iatrogenic with warfarin
  • Causes deficiency of Vit K dependent coagulation factors (Factors II, VII, IX, X)
  • Diagnosis: elevated PT, if severe may have prolonged PTT as well
  • Vitamin K deficiency will correct in the lab in a mixing study
  • Management: Replace vitamin K, see "Transfusion" section for more details