Bleeding Disorder

Aka: Bleeding Disorder, Bleeding Diathesis, Abnormal Bleeding

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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. Pathophysiology

  1. See Clotting Pathway

IV. History: Bleeding or Bruising History

  1. Screening Questions for congenital Bleeding Disorder
    1. ISTH Bleeding Assessment Tool (ISTH-BAT)
  2. Bleeding or Bruising history
    1. Source of bleeding (e.g. skin Bruising, Epistaxis, GI Bleed)
    2. Severity of bleeding (e.g. degree of bleeding, size of Bruising)
    3. Duration of bleeding until controlled
    4. Triggers (e.g. Trauma, dental procedure, Vaginal Delivery)
    5. Menorrhagia history
      1. Very heavy bleeding? (e.g. gushing, changing a saturated pad every 2 hours, large clots)
      2. Prolonged Menses (e.g. >=8 days)
      3. Onset of heavy Menses after age 20 years? (inherited disorder before age 20, acquired after age 20)
    6. Spontaneous bleeding episodes
      1. Coagulation Disorders: Spontaneous hemarthroses (bloody joint effusions) and muscle Hematomas
      2. Platelet Disorders: Spontaneous mucocutaneous bleeding (e.g. Bleeding Gums, Epistaxis)
  3. Red flags suggestive of significant Bleeding Disorder
    1. Bleeding from >=2 mucocutaneous sites
    2. Epistaxis episodes >5 per year OR one episode lasting >10 minutes
    3. Bleeding duration >10 minutes after a minor Laceration
    4. Heavy and prolonged Menstrual Bleeding
    5. Unexplained Postpartum Hemorrhage
    6. Bleeding episodes >3 from a single site
    7. Bleeding episode requiring Blood Transfusion

V. History: Past Medical History

  1. 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
  2. Medications and Substances
    1. See Anticoagulant
    2. See Drug Induced Platelet Dysfunction
    3. Alcohol Use Disorder
  3. Dietary history
    1. Restrictive diets may result in Vitamin C or Vitamin K Deficiency

VI. History: Symptoms or Clinical Clues

  1. Age of onset
    1. Young age
      1. Severe inherited Bleeding Disorders are typically diagnosed in the first few years of life
      2. Consider in excessive bleeding from Circumcision, umbilical stump, large or numerous Bruises
      3. All U.S. newborns are given IM Vitamin K (unless refused) to prevent Hemorrhagic Disease of the Newborn
    2. Teens
      1. Von Willebrand Disease diagnosis is often delayed until Menarche (teen girls)
    3. Older age
      1. Thinning of skin and subcutaneous tissue, and capillary weakening leads to Bruising in older adults
  2. Critical Illness or hospitalization
    1. Thrombotic Thrombocytopenic Purpura
    2. Disseminated Intravascular Coagulation
  3. Acute Diarrhea (E. coli 0157:H7)
    1. Hemolytic Uremic Syndrome
  4. Upper Respiratory Infection (esp. Streptococcal Pharyngitis)
    1. Henoch Schonlein Purpura
  5. 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)
  6. Night Sweats, fever and weight loss (B Symptoms)
    1. Hematologic Malignancy (Leukemia, Myelodyspastic syndrome, Lymphoma)
  7. Malnutrition
    1. Alcoholic Liver Disease (Alcoholic Cirrhosis)
    2. Vitamin C Deficiency
    3. Vitamin K Deficiency (esp. infants <6 months who did NOT receive as newborn)
  8. 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 Forearms)
    4. Coagulation Disorders
      1. Associated with spontaneous hemarthroses (bloody joint effusions) and muscle Hematomas
  9. Pregnancy
    1. HELLP Syndrome
      1. Severe Preeclampsia with Hemolysis, elevated liver enzymes and Low Platelet Count

VII. 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 Cephalohematomas, 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

VIII. 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. Dcoument size, number, and location of lesions (and consider images for medical record)
    2. Lesions
      1. Petechiae (<2 mm)
      2. Purpura (2 to 10 mm)
      3. Ecchymosis (>10 mm)
      4. Telangiectasias
    3. Red Flags suggestive of Bleeding Disorder
      1. Truncal Bruising
      2. Five or more Ecchymosis (>10 mm)
  6. Trauma
    1. Excessive bleeding from minor wounds
    2. Excessive bleeding following surgery or dental procedures
    3. Intracerebral bleeding event
    4. Consider physical abuse
      1. See Non-Accidental Trauma in Children
      2. Elder Abuse
        1. Consider in large Bruises (>5 cm), and Bruising to face, lateral right arm or posterior torso

IX. 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
  5. Joint Hyperextensibility
    1. Marfan Syndrome
    2. Ehlers-Danlos Syndrome (EDS)

X. Causes

  1. Coagulation Disorder
    1. See Coagulation Bleeding Disorders
    2. See Coagulopathy in Pregnancy
  2. Platelet Disorders
    1. See Platelet Bleeding Disorders
    2. See Thrombocytopenia
    3. See Drug Induced Platelet Dysfunction
  3. Vascular Disorders
    1. See Blood Vessel Wall Bleeding Disorders

XI. Labs: Initial

  1. Complete Blood Count with Platelets
  2. Peripheral Blood Smear
  3. ProTime (PT) with INR
  4. Activated Partial Thromboplastin Time (aPTT)
  5. Fibrinogen
  6. Comprehensive metabolic panel (Liver Function Tests and Renal Function tests)
  7. Bleeding Time is NOT typically used due to lack of standardization

XII. Evaluation: Based on initial testing

  1. See ISTH Bleeding Assessment Tool (ISTH-BAT)
  2. 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
  3. 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
        1. Hemophilia A (Factor VIII Deficiency, 85% of Hemophilia cases)
        2. Hemophilia B (Factor IX Deficiency, 15% of Hemophilia cases)
        3. Hemophilia C (Factor XI Deficiency, rare)
      2. Consider Von Willebrand's testing if low Factor VIII
        1. Von Willebrand Disease alone does not affect PTT
        2. Von Willebrand Disease with Factor VIII:C deficiency results in a mild increase in PTT
    2. PTT does not correct with a PTT Mixing Study (mixed with normal blood)
      1. Obtain Lupus Anticoagulant
      2. Obtain Factor VIII Inhibitor
  4. ProTime (PT) or INR prolonged 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
      2. Also consider liver disease, early Disseminated Intravascular Coagulation
      3. Confirm not taking a Vitamin K Antagonist (e.g. Warfarin)
  5. BOTH ProTime (PT/INR) and Partial Thromboplastin Time (PTT) Abnormal
    1. Causes
      1. Comorbid advanced liver disease (e.g. Cirrhosis)
      2. Disseminated Intravascular Coagulation (DIC)
      3. Anticoagulant use (e.g. Warfarin, Heparin, Direct Thrombin Inhibitors)
      4. Common Clotting Pathway Disorder
        1. Factor X Deficiency (may also occur in Amyloidosis)
        2. Factor V Deficiency
        3. Factor II Deficiency (Prothrombin)
        4. Factor I Deficiency (Fibrinogen Deficiency)
    2. Labs
      1. Liver Function Tests
      2. Fibrinogen level
      3. Coagulation Factor Assays
  6. Platelet abnormality
    1. See Platelet Bleeding Disorders
    2. See Drug Induced Platelet Dysfunction
    3. See Thrombocytopenia
    4. Peripheral Blood Smear for microscopic Platelet abnormalities
    5. Platelet function tests (specialty lab typically ordered by hematology)
      1. Light transmission aggregometry (or, if not available, then PFA 100)
      2. Avoid Bleeding Time (low sensitivity and lack of standardization)
  7. Images
    1. ThrombosisPathway.png

XIII. Management

  1. Hemorrhage Management
    1. See Hemorrhage Management
    2. See Emergent Reversal of Anticoagulation
  2. Manage Specific Conditions
    1. See Hemophilia A (Factor VIII Deficiency)
    2. See Hemophilia B (Factor IX Deficiency)
    3. See Von Willebrand Disease
  3. Consider Acquired Coagulopathy
    1. Cirrhosis (decrease of both coagulant and Anticoagulant factors)
    2. End Stage Renal Disease (Anemia, Platelet Dysfunction)
    3. Rattlesnake bite (Fibrinogen deficiency)
    4. Massive Transfusion (Dilutional Coagulopathy)
    5. Disseminated Intravascular Coagulation (DIC)
      1. Constant oozing of blood from inserted lines, drains and tubes
      2. Multiple sites of Hemorrhage including Gastrointestinal Bleeding
  4. 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

XIV. References

  1. Ferdjallah (2024) Mayo Clinic Pediatric Days, attended lecture 1/16/2024
  2. Allen (2002) Pediatr Clin North Am 49: 1239-56 [PubMed]
  3. Ballas (2008) Am Fam Physician 77:1117-24 [PubMed]
  4. Hughes (2024) Am Fam Physician 110(5): 504-14 [PubMed]
  5. Jones (2024) Am Fam Physician 110(1): 58-64 [PubMed]
  6. Neutze (2016) Am Fam Physician 93(4): 279-86 [PubMed]

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