Coagulopathies

• 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