Rosen & Barkin's 5-Minute Emergency Medicine Consult (325 page)

• Hemarthrosis:
  
  • Splint, ace, ice
    
  • Arthrocentesis is rarely indicated
    
• Muscle hemorrhage:
  
  • Forearm/calf—consider compartment syndrome
    
  • Psoas hematoma—groin pain, femoral nerve paresthesias
    
• Post tooth extraction or oral mucosal bleeding:
  
  • Treat locally with collagen sponge (Avitene) or other hemostatic agent.
    
  • Replace factor if severe.
    
  • Aminocaproic acid (Amicar) or tranexamic acid (Cyklokapron) may be useful.
    
• Hematuria (generally mild):
  
  • Hydrate
    
  • Avoid Amicar and cryoprecipitate.
    
• Intracranial hemorrhage:
  
  • All head injuries should be considered significant, especially in children.
    
  • Do not delay therapy for CT head.
    
• GI bleeding:
  
  • Secondary to ulcers, polyps, hemorrhoids
    
  • Replace factor prior to endoscopy.
    

To calculate doses for recombinant and plasma-derived factor VIII and factor IX concentrates refer to section Approach to Factor Replacement above.

• First line: Recombinant factor
  
  • Recombinant factor VIII options: Recombinate, Kogenate, Advate, Helixate FS, Xyntha
    
  • Recombinant factor IX options: BeneFIX
    
• Second line: Plasma-derived factor
  
  • Plasma-derived factor VIII high-purity concentrates: Monoclate-P, Hemofil-M, Koate DVI, Alphanate
    
  • Plasma-derived factor VIII intermediate-purity concentrates: Humate
    
    • Only use when previous options are not available.
      
  • Plasma-derived factor IX high-purity concentrates: AlphaNine, Mononine
    
• Other options
  
  • Cryoprecipitate (useful only in hemophilia A):
    
    • Obtained from FFP after thawing at 4°C
      
    • Contains factor VIII, vWF, fibrinogen
      
    • Estimated 80–100 U of factor VIII in 1 U of cryoprecipitate
      
    • Give 10 bags initially, peds dose 1 bag/6 kg
      
    • Only if factor VIII concentrates are not available
      
  • FFP (contains factors VIII and IX):
    
    • 1 U of FFP contains about 200–300 U each of both factor VIII and factor IX
      
    • 1 U of FFP will raise factor level 5–10% in a 60 kg person
      
    • Readily available in most EDs; useful in life-threatening bleeds when access to specific factor treatment is delayed
      
  • Adjunct to factor therapy: DDAVP (only in hemophilia A)
    
    • Raises factor VIII level 2–3 times (only use in patients with mild hemophilia, and only with mild bleeds)
      
    • Side effects: Mild flushing, headache, tachycardia, hypotension, hyponatremia
      
    • Dose 0.3 μg/kg of DDAVP diluted in 50 mL 0.9% NS given over 15–30 min given IV or SC (intranasal “Stimate” 1 spray each nostril if >50 kg)
      
    • Should not be used in children <1 yr old
      
  • Adjunct to factor therapy: Antifibrinolytic agents (for mild bleeds)
    
    • Inhibit plasmin activity, prevents clot lysis
      
    • Only for mucosa/oral/dental bleeding
      
    • Do not use in children.
      
    • Examples: Aminocaproic acid (Amikar) 75 mg/kg every 6 hr up to 4 g or tranexamic acid (Cyklokapron) 25 mg/kg every 8 hr.
      
  • Adjunct to factor therapy: Topical thrombin
    
    • Useful for localized control of bleeding from lacerated superficial tissues
      

• First line: Recombinant factor VIIa (NovoSeven)
  
  • 90–120 μg/kg every 2–3 hr
    
  • Higher doses of up to 300 μg/kg may promote thrombin formation and stabilize fibrin clot in the case of inefficient hemostasis
    
  • Bypasses the coagulation cascade and works locally, decreasing risk for systemic coagulation
    
  • Strong safety profile, minimal risk of inhibitor development, expensive
    
• Second line: Activated prothrombin complex concentrates (APCCs)
  
  • Examples: FEIBA and Autoplex-T
    
  • Dose: 75–100 U/kg every 8–12 hr
    
  • Contains VIIa, IXa, and Xa
    
  • Bypasses coagulation cascade
    
  • Thromboembolic events occur with higher doses: DVT, PE, acute MI, DIC
    
• Other options
  
  • Use high-dose recombinant or plasma-derived factor concentrates (2–3 times normal range) in those who have low antibody titers (low responders)
    
  • Porcine factor VIII is now off the market
    

FOLLOW-UP

• Low threshold for admission
  
• All joint/muscle bleeds, internal bleeding, severe bleeds
  
• Bleeding that may require multiple transfusions
  
• Severe complications or any head trauma
  

Minor soft tissue bleeding, superficial lacerations with resolution/control of bleeding

Hematology for all bleeds

• Hematologist as outpatient
  
• Return to ER for recurrent bleeding episodes
  

• Do not wait for a head CT before treatment in the setting of head trauma
  
• In a suspected bleeding emergency in a patient with unknown clotting factor level, assume the level to be 0 and treat as a severe bleed
  
• Be familiar with different factor replacement therapies for patients with and without inhibitors
  
• Consult hematologist for appropriate dosing in ER, length of treatment, inhibitor vs. noninhibitor treatment
  
• Have a low threshold for admission
  

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• Franchini M, Lippi G. The use of desmopressin in acquired haemophilia A: A systematic review.
  Blood Transfus.
  2011;9:377–382.
  
• Hay CR, Brown S, Collins PW, et al. The diagnosis and management of factor VIII and IX inhibitors: A guideline from the United Kingdom Haemophilia Centre Doctors Organization.
  Br J Haematol.
  2006;133:591–605.
  
• Kempton CL, White GC 2nd. How we treat a hemophilia A patient with a factor VIII inhibitor.
  Blood
  . 2009;113:11–17.
  
• Key NS, Negrier C. Coagulation factor concentrates: Past, present, future.
  Lancet
  . 2007;370:439–448.
  
• Pipe SW. Recombinant clotting factors.
  Thromb Haemost
  . 2008;99:840–850.
  
• Singleton T, Kruse-Jarres R, Leissinger C. Emergency Department care for patients with hemophilia and von Willibrand disease.
  J Emerg Med
  . 2010;39(2):158–165.
  

CODES

• 286.0 Congenital factor VIII disorder
  
• 286.1 Congenital factor IX disorder
  

BASICS

• Expectoration of blood originating from the tracheobronchial tree
  
• Source of bleeding:
  
  • Bronchial arteries (90%), usually causes profuse bleeding
    
  • Pulmonary arteries (5%), usually causes small amounts of bleeding
    
  • Nonbronchial arteries (5%) including intercostal arteries, coronary arteries, thoracic, upper, and inferior phrenic arteries
    
• Threshold of massive hemoptysis defined has been defined from 100 mL to 1 L/24 hr:
  
  • >8 mL/kg/day in children
    
  • Most common definition is >300–600 mL/24 hr
    
• Mortality:
  
  • Massive hemoptysis (>500 mL/24 hr): 38%
    
  • Trivial to moderate hemoptysis (<500 mL/24 hr): 4.5%
    
  • Malignancy and coagulopathy increase the risk of mortality
    

• Infectious (most common cause):
  
  • Acute or chronic bronchitis
    
  • Pneumonia
    
  • Necrotizing pneumonia or lung abscess (
    Klebsiella pneumoniae, Pseudomonas aeruginosa, Staphylococcus aureus, Streptococcus pneumoniae
    )
    
  • Tuberculosis
    
  • Viral (influenza, varicella)
    
  • Fungal (Aspergillus
    ,
    Coccidioides
    ,
    Histoplasma
    ,
    Blastomyces)
    
  • Parasitic (Ascariasis, Amebiasis, Paragonimiasis, Echinococcus)
    
• Neoplastic:
  
  • Squamous cell, small cell, carcinoid
    
  • Bronchogenic carcinoma
    
  • Metastatic disease
    
• Pulmonary:
  
  • Bronchiectasis
    
  • Pulmonary embolism/infarction
    
  • Cystic fibrosis
    
  • Bronchopleural fistula
    
  • Sarcoidosis
    
• Cardiac:
  
  • Mitral stenosis
    
  • Tricuspid endocarditis
    
  • Heart failure
    
• Systemic disease:
  
  • Goodpasture syndrome
    
  • Systemic lupus erythematosus
    
  • Vasculitis (Wegener granulomatosis, Henoch–Schönlein purpura, Behcçet disease)
    
• Hematologic:
  
  • Coagulopathy
    
  • Thrombocytopenia
    
  • Platelet dysfunction
    
  • DIC
    
• Vascular:
  
  • Pulmonary HTN
    
  • Arteriovenous malformation
    
  • Aortic aneurysm
    
  • Pulmonary artery aneurysm (Rasmussen aneurysm, mycotic, arteritis)
    
  • Aortobronchial fistula
    
• Drugs/toxins:
  
  • Aspirin/antiplatelet therapy
    
  • Anticoagulants
    
  • Penicillamine, amiodarone, propylthiouracil, bevacizumab
    
  • Cocaine (“crack”) lung
    
  • Organic solvents
    
• Trauma:
  
  • Tracheobronchial rupture
    
  • Pulmonary contusion
    
• Iatrogenic:
  
  • Bronchoscopy/lung biopsy
    
  • Pulmonary artery or central venous catheterization
    
  • Transtracheal aspirate
    
• Miscellaneous:
  
  • Foreign-body aspiration
    
  • Catamenial hemoptysis (pulmonary endometriosis)
    
  • Amyloidosis
    
  • Idiopathic or cryptogenic (between 5% and 30%, depending on patient population)