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Bleeding Disorder
Aka: Bleeding Disorder, Bleeding Diathesis, Abnormal Bleeding
- See Also
- Clotting Pathway
- ISTH Bleeding Assessment Tool (ISTH-BAT)
- Epidemiology
- Bleeding Gums, Epistaxis and easy bruisability are common in healthy patients (occurs in up to 45%)
- Menorrhagia is very common in women (5-10%)
- Bleeding Disorders contribute to up to 29% of Menorrhagia cases (esp. Von Willebrand Disorder)
- History: Symptoms or clinical clues
- Critical Illness or hospitalization
- Thrombotic Thrombocytopenic Purpura
- Disseminated Intravascular Coagulation
- Acute Diarrhea (E. coli 0157:H7)
- Hemolytic Uremic Syndrome
- Upper Respiratory Infection (esp. Streptococcal Pharyngitis)
- Henoch Schonlein Purpura
- Chronic Bleeding Disorder
- Systemic Lupus Erythematosus
- Ehlers-Danlos Syndrome
- Hypermobile joints
- Von Willebrand Disease
- Menorrhagia (most common), recurrent Epistaxis or Gingival Bleeding
- Often delayed diagnosis with normal basic coagulation labs (until Platelet closure time is checked)
- Hemophilia A (Factor VIII) or Hemophilia B (Factor IX) deficiency
- Hemoarthrosis or other soft tissue bleeding in males
- Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome)
- Telangiectasias involving lips, Tongue, skin, nose and GI Tract
- Immune Thrombocytopenic Purpura
- Especially children (often following viral syndrome such as EBV, VZV or CMV)
- Night Sweats and weight loss
- Hematologic Malignancy (Leukemia, Myelodyspastic syndrome, Lymphoma)
- Malnutrition
- Alcoholic Liver Disease (Alcoholic Cirrhosis)
- Vitamin C Deficiency
- Vitamin K Deficiency
- Bruising
- Physical Abuse
- Purpura Simplex
- Women with Bruising on arms and upper thighs
- Senile Purpura
- Older adults with dark Bruises in areas of thin skin (esp. extensor arms)
- Pregnancy
- HELLP Syndrome
- Severe Preeclampsia with Hemolysis, elevated liver enzymes and Low Platelet Count
- History
- Screening Questions for congenital Bleeding Disorder
- ISTH Bleeding Assessment Tool (ISTH-BAT)
- Family History of Bleeding Disorder (e.g. Hemophilia, Von Willebrand Disease)
- Include second-degree relatives, and back several generations
- Negative Family History does not exclude inherited Bleeding Disorder
- Medications
- See Anticoagulant
- See Drug Induced Platelet Dysfunction
- Signs: Abnormal Bleeding (multiple sites)
- Nasopharynx
- Epistaxis
- Bleeding Gums
- Gastrointestinal
- Hematemesis
- Melana
- Gynecologic
- Menorrhagia
- Postpartum Hemorrhage
- Musculoskeletal
- Muscle Hematomas
- Hemarthrosis
- Skin
- Purpura
- Petechiae
- Trauma
- Excessive bleeding from minor wounds
- Excessive bleeding following surgery or dental procedures
- Intracerebral bleeding event
- Signs: Clinical Clues
- Spontaneous hemarthrosis, muscle Hemorrhage or retroperitoneal bleeding
- Congenital Bleeding Disorder
- Mucocutaneous bleeding (Petechiae, Epistaxis, Gingival Bleeding, GI Bleeding, GU Bleeding)
- Platelet Bleeding Disorder
- Hepatomegaly
- Liver disorder
- Splenomegaly
- Hematologic Malignancy
- Idiopathic Thrombocytopenic Purpura
- Causes
- Coagulation Disorder
- See Coagulation Bleeding Disorders
- See Coagulopathy in Pregnancy
- Platelet Disorders
- See Platelet Bleeding Disorders
- See Thrombocytopenia
- See Drug Induced Platelet Dysfunction
- Vascular Disorders
- See Blood Vessel Wall Bleeding Disorders
- Labs: Initial
- Complete Blood Count with Platelets
- Peripheral Blood Smear
- ProTime (PT) with INR
- Partial Thromboplastin Time (PTT)
- Comprehensive metabolic panel (Liver Function Tests and Renal Function tests)
- Labs: Based on initial testing
- Normal PT, PTT, and Platelet Count/morphology
- Obtain labs
- Von Willebrand Factor Antigen
- Von Willebrand Factor Activity (risocetin Cofactor activity)
- Factor VIII Level
- AVOID Platelet Function Closure Time (PFCT, Platelet Function Analyzer-100)
- No longer recommended due to False Negatives in less than severe cases of Von Willebrand
- Abnormal labs
- Von Willebrand Disease (additional testing can identify type)
- Normal labs
- Refer to hematology for additional evaluation of Platelet function disorder
- May require light transmission aggregometry
- Partial Thromboblastin Time (PTT) abnormality and Normal PT/INR (Intrinsic Clotting Pathway Abnormal)
- PTT corrects with a PTT Mixing Study (patient plasma mixed 1:1 with normal plasma)
- Obtain Factor VIII, Factor IX, and Factor XI assays
- Consider Von Willebrand's testing if low Factor VIII
- PTT does not correct with a PTT Mixing Study (mixed with normal blood)
- Obtain Lupus Anticoagulant
- Obtain Factor VIII Inhibitor
- ProTime (PT) or INR abnormal and Normal PTT (Extrinsic Clotting Pathway Abnormal) - uncommon
- PT/INR corrects with Vitamin K Supplementation
- Replace Vitamin K as needed
- Assess for Malnutrition and malabsorption causes of Vitamin K Deficiency
- PT/INR does not correct with Vitamin K Supplementation
- Obtain Factor VII assay
- BOTH ProTime (PT/INR) and Partial Thromboplastin Time (PTT) Abnormal
- Causes
- Comorbid advanced liver disease
- Disseminated Intravascular Coagulation (DIC)
- Anticoagulant use (Warfarin or Heparin)
- Combined Intrinsic Clotting Pathway and Extrinsic Clotting Pathway
- Labs
- Liver Function Tests
- Fibrinogen level
- Factor Assays
- Platelet abnormality
- See Platelet Bleeding Disorders
- See Drug Induced Platelet Dysfunction
- See Thrombocytopenia
- Management: Hematology Consultation indications
- Significant finding on testing
- Nondiagnostic results with high clinical suspicion
- Preoperative concern for Bleeding Disorder
- References
- Allen (2002) Pediatr Clin North Am 49: 1239-56 [PubMed]
- Ballas (2008) Am Fam Physician 77:1117-24 [PubMed]
- Neutze (2016) Am Fam Physician 93(4): 279-86 [PubMed]