II. Epidemiology

  1. Bleeding Gums, Epistaxis and easy bruisability are common in healthy patients (occurs in up to 45%)
  2. Menorrhagia is very common in women (5-10%)
    1. Bleeding Disorders contribute to up to 29% of Menorrhagia cases (esp. Von Willebrand Disorder)

III. History: General

  1. Screening Questions for congenital Bleeding Disorder
    1. ISTH Bleeding Assessment Tool (ISTH-BAT)
  2. Family History of Bleeding Disorder (e.g. Hemophilia, Von Willebrand Disease)
    1. Include second-degree relatives, and back several generations
    2. Negative Family History does not exclude inherited Bleeding Disorder
  3. Medications
    1. See Anticoagulant
    2. See Drug Induced Platelet Dysfunction

IV. History: Symptoms or clinical clues

  1. Critical Illness or hospitalization
    1. Thrombotic Thrombocytopenic Purpura
    2. Disseminated Intravascular Coagulation
  2. Acute Diarrhea (E. coli 0157:H7)
    1. Hemolytic Uremic Syndrome
  3. Upper Respiratory Infection (esp. Streptococcal Pharyngitis)
    1. Henoch Schonlein Purpura
  4. Chronic Bleeding Disorder
    1. Systemic Lupus Erythematosus
    2. Ehlers-Danlos Syndrome
      1. Hypermobile joints
    3. Von Willebrand Disease
      1. Menorrhagia (most common), recurrent Epistaxis or Gingival Bleeding
      2. Often delayed diagnosis with normal basic coagulation labs (until Platelet closure time is checked)
    4. Hemophilia A (Factor VIII) or Hemophilia B (Factor IX) deficiency
      1. Hemoarthrosis or other soft tissue bleeding in males
    5. Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome)
      1. Telangiectasias involving lips, Tongue, skin, nose and GI Tract
    6. Immune Thrombocytopenic Purpura
      1. Especially children (often following viral syndrome such as EBV, VZV or CMV)
  5. Night Sweats and weight loss
    1. Hematologic Malignancy (Leukemia, Myelodyspastic syndrome, Lymphoma)
  6. Malnutrition
    1. Alcoholic Liver Disease (Alcoholic Cirrhosis)
    2. Vitamin C Deficiency
    3. Vitamin K Deficiency
  7. Bruising
    1. Physical Abuse
    2. Purpura Simplex
      1. Women with Bruising on arms and upper thighs
    3. Senile Purpura
      1. Older adults with dark Bruises in areas of thin skin (esp. extensor arms)
  8. Pregnancy
    1. HELLP Syndrome
      1. Severe Preeclampsia with Hemolysis, elevated liver enzymes and Low Platelet Count

V. History: Example Presentations

  1. Excessive Newborn umbilical stump bleeding
    1. Associated disorders include coagulation Protein deficits, Factor XIII deficiency
    2. Occult Intracranial Hemorrhage may be associated
  2. Male infant with swollen joints
    1. Common presentation in Hemophilia
    2. May be associated with forehead Hematomas, excessive post-Circumcision bleeding
  3. Post-viral syndrome in a previously healthy child
    1. Associated with Immune Thrombocytopenic Purpura
    2. May present with Petechiae or oral Purpura
  4. Adolescent females with heavy Menorrhagia
    1. Common presentation in Von Willebrand Disease
    2. May be associated with recurrent Epistaxis, severe Iron Deficiency Anemia

VI. Signs: Abnormal Bleeding (multiple sites)

  1. Nasopharynx
    1. Epistaxis
    2. Bleeding Gums
  2. Gastrointestinal
    1. Hematemesis
    2. Melana
  3. Gynecologic
    1. Menorrhagia
    2. Postpartum Hemorrhage
  4. Musculoskeletal
    1. Muscle Hematomas
    2. Hemarthrosis
  5. Skin
    1. Purpura
    2. Petechiae
  6. Trauma
    1. Excessive bleeding from minor wounds
    2. Excessive bleeding following surgery or dental procedures
    3. Intracerebral bleeding event

VII. Signs: Clinical Clues

  1. Spontaneous hemarthrosis, muscle Hemorrhage or retroperitoneal bleeding
    1. Congenital Bleeding Disorder
  2. Mucocutaneous bleeding (Petechiae, Epistaxis, Gingival Bleeding, GI Bleeding, GU Bleeding)
    1. Platelet Bleeding Disorder
  3. Hepatomegaly
    1. Liver disorder
  4. Splenomegaly
    1. Hematologic Malignancy
    2. Idiopathic Thrombocytopenic Purpura

IX. Labs: Initial

X. Labs: Based on initial testing

  1. Normal PT, PTT, and Platelet Count/morphology
    1. Obtain labs
      1. Von Willebrand Factor Antigen
      2. Von Willebrand Factor Activity (risocetin Cofactor activity)
      3. Factor VIII Level
      4. AVOID Platelet Function Closure Time (PFCT, Platelet Function Analyzer-100)
        1. No longer recommended due to False Negatives in less than severe cases of Von Willebrand
    2. Abnormal labs
      1. Von Willebrand Disease (additional testing can identify type)
    3. Normal labs
      1. Refer to hematology for additional evaluation of Platelet function disorder
      2. May require light transmission aggregometry
  2. Partial Thromboblastin Time (PTT) abnormality and Normal PT/INR (Intrinsic Clotting Pathway Abnormal)
    1. PTT corrects with a PTT Mixing Study (patient plasma mixed 1:1 with normal plasma)
      1. Obtain Factor VIII, Factor IX, and Factor XI assays
      2. Consider Von Willebrand's testing if low Factor VIII
    2. PTT does not correct with a PTT Mixing Study (mixed with normal blood)
      1. Obtain Lupus Anticoagulant
      2. Obtain Factor VIII Inhibitor
  3. ProTime (PT) or INR abnormal and Normal PTT (Extrinsic Clotting Pathway Abnormal) - uncommon
    1. PT/INR corrects with Vitamin K Supplementation
      1. Replace Vitamin K as needed
      2. Assess for Malnutrition and malabsorption causes of Vitamin K Deficiency
    2. PT/INR does not correct with Vitamin K Supplementation
      1. Obtain Factor VII assay
  4. BOTH ProTime (PT/INR) and Partial Thromboplastin Time (PTT) Abnormal
    1. Causes
      1. Comorbid advanced liver disease
      2. Disseminated Intravascular Coagulation (DIC)
      3. Anticoagulant use (Warfarin or Heparin)
      4. Combined Intrinsic Clotting Pathway and Extrinsic Clotting Pathway
    2. Labs
      1. Liver Function Tests
      2. Fibrinogen level
      3. Factor Assays
  5. Platelet abnormality
    1. See Platelet Bleeding Disorders
    2. See Drug Induced Platelet Dysfunction
    3. See Thrombocytopenia

XI. Management: Hematology Consultation indications

  1. Significant finding on testing
  2. Preoperative concern for Bleeding Disorder
    1. Prior history of excessive bleeding with invasive procedures
  3. Nondiagnostic results with high clinical suspicion
  4. Major or excessive bleeding with minor Trauma

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