Evaluation of Bleeding Tendencies

Evaluation of Bleeding Tendencies

Evaluation of Bleeding Tendencies

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Initial Assessment - Bleed Detectives

  • Focused History:
    • Onset & Type: Spontaneous vs. post-trauma/surgery? Neonatal onset?
    • Sites: Mucocutaneous (epistaxis, gum bleed, menorrhagia) vs. deep tissue (hemarthrosis, muscle hematoma).
    • Severity & Frequency: Quantify if possible.
    • Family History: Bleeding disorders? Consanguinity?
    • Drug History: NSAIDs, anticoagulants.
    • Systemic Illness: Liver/renal disease, infections.
  • Clinical Examination:
    • Skin/Mucosa: Petechiae (<2mm), purpura (2mm-1cm), ecchymoses (>1cm), hematomas. Petechiae, purpura, and ecchymoses on pediatric skin
    • Joints: Hemarthrosis (swelling, pain, restricted movement).
    • General: Pallor, jaundice, hepatosplenomegaly.

⭐ Mucocutaneous bleeding (gums, nose, GI, menorrhagia) suggests platelet disorders or von Willebrand disease; deep tissue bleeding (joints, muscles, CNS) suggests coagulation factor deficiencies.

Lab Investigations - Initial Clues

  • Initial Screening Tests:
    • CBC: Platelet count.
    • PT: Extrinsic & common pathways.
    • aPTT: Intrinsic & common pathways.
    • BT/PFA-100: Platelet function, vWD screen.

⭐ A mixing study (patient plasma + normal plasma) helps differentiate factor deficiency (correction of PT/aPTT) from a factor inhibitor (no correction).

Primary Hemostasis Disorders - Plug Problems

Mucocutaneous bleeds (petechiae, epistaxis). Labs: PT/aPTT normal, ↑BT/PFA-100.

  • Immune Thrombocytopenic Purpura (ITP)

    • Patho: Anti-GpIIb/IIIa Abs → ↓platelets.
    • Features: Isolated ↓Plt, post-viral (kids).
    • BM: ↑Megakaryocytes.
  • von Willebrand Disease (vWD)

    • Patho: vWF def./defect (binds FVIII).
    • Labs: ↑BT/PFA, ↓vWF:Ag, ↓Risto. Cof; Plt N/↓; aPTT often ↑.

    ⭐ von Willebrand Disease is the most common inherited bleeding disorder, typically presenting with mucocutaneous bleeding and often a normal platelet count but prolonged PFA-100/Bleeding Time.

  • Bernard-Soulier Syndrome (BSS)

    • Patho: GpIb defect (adhesion); AR.
    • Features: Thrombocytopenia, GIANT platelets.
    • Labs: ↓Plt (giant), ↑BT. No Ristocetin agg.
  • Glanzmann Thrombasthenia (GT)

    • Patho: GpIIb/IIIa defect (aggregation); AR.
    • Features: Normal platelet count.
    • Labs: Normal Plt, ↑BT. No ADP/collagen/epi agg.
  • Drug-induced Platelet Dysfunction

    • Aspirin (irreversible), NSAIDs (reversible): ↓TXA2 → impaired aggregation.

Secondary Hemostasis Disorders - Factor Fumbles

Coagulation Cascade Diagram

  • Hemophilia A: Factor VIII↓. X-linked recessive. Deep bleeds (joints, muscles). Lab: ↑aPTT; Normal PT, Platelet count, BT.
  • Hemophilia B (Christmas Disease): Factor IX↓. X-linked recessive. Clinically like Hemophilia A. Lab: ↑aPTT; Normal PT, Platelet count, BT.

⭐ Hemophilia A (Factor VIII deficiency) and Hemophilia B (Factor IX deficiency) are X-linked recessive disorders characterized by prolonged aPTT with normal PT and platelet count.

  • Vitamin K Deficiency: Factors II, VII, IX, X, Protein C, S↓. Acquired (e.g., antibiotics, warfarin). Lab: ↑PT (early, due to FVII short half-life), then ↑aPTT.
  • Von Willebrand Disease (vWD): vWF↓ (stabilizes FVIII). Autosomal Dominant (most common type). Mucocutaneous bleeding. Lab: Often ↑BT, may have ↑aPTT (due to FVIII↓). Normal PT, Platelet count usually normal.

High‑Yield Points - ⚡ Biggest Takeaways

  • Initial screening: CBC (platelets), PT, aPTT are crucial first steps.
  • Isolated thrombocytopenia often suggests Immune Thrombocytopenic Purpura (ITP).
  • Prolonged aPTT with normal PT points to Hemophilia A/B or vWD (severe).
  • Von Willebrand Disease (vWD) is the most common inherited bleeding disorder; presents variably.
  • Mixing studies differentiate factor deficiency (corrects) from inhibitors (no correction).
  • Vitamin K deficiency classically prolongs PT more than aPTT.
  • Platelet function defects (e.g., Glanzmann's) show normal platelet count but abnormal function tests.
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