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Jason Jones


  • Step one: determine if 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; tendency 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
  • Consult benign hematology every time these patients are admitted

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.
  • Type 3 (rare): 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; most patients are treated with factor concentrate replacements


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 always (rare disorder with major bleeding complications)

Vitamin K Deficiency

  • Caused by malnutrition, liver disease, or iatrogenic with warfarin
  • Diagnosis: elevated PT, if severe may have prolonged PTT as well
  • Management: Replace vitamin K

Last update: 2022-06-25 02:05:01