Rosen & Barkin's 5-Minute Emergency Medicine Consult (710 page)

TREATMENT

• ABCs
  
• Evaluate for other possible causes of altered mental status (hypoglycemia, overdose)
  

• ABCs
  
• 0.9% normal saline (NS) IV fluid resuscitation for shock or GI hemorrhage
  
• RBC transfusions:
  
  • For significant anemia or bleeding complications
    
• Platelet transfusions:
  
  • Reserve for life-threatening hemorrhage (e.g., CNS bleeds) or required invasive procedures
    
  • May aggravate the thrombotic, microvascular obstructive process and worsen the end-organ ischemia and shock
    

• Fresh frozen plasma
  (FFP) or fresh unfrozen plasma:
  
  • Initiated as bridge to exchange transfusions on diagnosis of TTP
    
  • Success rate approaching 64%
    
  • Provides a platelet-antiaggregating factor absent or diminished in patient’s own serum
    
  • Used prophylactically to prevent recurrence in chronic relapsing variant
    
• Plasma exchange transfusions:
  
  • Most important component of treatment
    
  • Combination of plasmapheresis and FFP infusion
    
  • Plasmapheresis removes:
    
    • Immune complexes responsible for endothelial damage and initiation of TTP
      
    • Circulating proaggregation factors promoting platelet aggregation
      
  • Perform daily until:
    
    • Platelet count normalizes
      
    • Neurologic symptoms improve
      
    • LDH normalizes
      
  • Improvement of renal function may lag behind other findings.
    
  • Taper frequency based on empiric judgment of response; may need to resume if relapse occurs.
    
  • Complications include:
    
    • Allergy or serum sickness
      
    • Secondary infection
      
    • Hypotension
      
• Corticosteroids:
  
  • Unproven therapeutic benefit
    
  • May limit immunologically mediated endothelial damage and decrease splenic sequestration of platelets and damaged RBCs
    
  • Supportive benefit if adrenal glands damaged through hemorrhage or ischemia
    
• Antiplatelet or immunosuppressive drugs:
  
  • Aspirin and dipyridamole most commonly used
    
  • Use of sulfapyrazine, dextran, and vincristine has been reported.
    
  • Used with variable effectiveness
    
  • Can worsen bleeding complications
    
• Splenectomy:
  
  • Historically recommended
    
  • Of uncertain efficacy
    
• Dialysis:
  
  • For renal failure
    

• Aspirin: 325–650 mg PO q4–6h
  
• Dipyridamole: 75–100 mg PO QID
  
• FFP:
  
  • Plasma infusion: 30 mL/kg/d (75–100 mL/h)
    
  • Plasma exchange transfusion: 3–4 L/d
    
• Methylprednisolone: 0.75 mg/kg q12h
  
• Prednisone: 1–2 mg/kg/d (high dose up to 200 mg/d)
  
• Rituximab: 375 mg/m
  ²
  IV once per week for 4–8 doses
  
• Vincristine: 1.4 mg/m
  ²
  once per week IV
  

FOLLOW-UP

• Newly diagnosed serious platelet disorder, especially with bleeding complications or altered mental status or renal dysfunction
  
• ICU admission for TTP with active bleeding or neurologic findings:
  
  • Transport to tertiary care center with appropriate specialty care facilities.
    

Patients with known disease and found to be stable may follow up with a hematologist.

• TTP can be confused with HELLP syndrome in pregnant females.
  
• Because of the high mortality of untreated TTP, recognition of the disease and initiation of treatment is key.
  

• George JN. Clinical practice. Thrombotic thrombocytopenic purpura.
  N Engl J Med
  . 2006;354:1927–1935.
  
• George JN. How I treat patients with thrombotic thrombocytopenic purpura: 2010.
  Blood
  2010;116:4060–4069.
  
• George JN, Woodson RD, Kiss JE, et al. Rituximab therapy for thrombotic thrombocytopenic purpura: A proposed study of the Transfusion Medicine/Hemostasis Clinical Trials Network with a systematic review of rituximab therapy for immune-mediated disorders.
  J Clin Apher
  . 2006;21:49–56.
  
• Kremer Hovinga JA, Meyer SC. Current management of thrombotic thrombocytopenic purpura.
  Curr Opin Hematol
  . 2008;15(5):445–450.
  

See Also (Topic, Algorithm, Electronic Media Element)

• Disseminated Intravascular Coagulation
  
• HELLP Syndrome
  
• Idiopathic Thrombocytopenia
  
• Renal Failure
  

CODES

446.6 Thrombotic microangiopathy

BASICS

• Distal phalangeal fractures:
  
  • Blunt trauma, hyperextension of the thumb, axial loading of the thumb, and crush injuries.
    
  • Tuft fracture
    is a similar fracture in other digits, in which the distal phalanx is crushed and/or fragmented.
    
  • It may be open or closed and associated with nail bed injury.
    
  • Severe nail bed injury, intra-articular, displaced/angulated fractures, or tendon injuries warrant orthopedics’ consultation.
    
  • Noncomplex tuft fractures can be splinted and treated as soft tissue injuries.
    
• Proximal phalangeal fractures and thumb metacarpal fractures:
  
  • Blunt trauma to the thumb:
    
    • Axial loading of the thumb with the metacarpophalangeal (MP) joint partially flexed, the hand closed or the thumb MP joint otherwise stabilized
      
  • Bennett fracture (type I):
    
    • Intra-articular fracture/dislocation at the base of the metacarpal where the ulnar aspect of the metacarpal maintains its attachment.
      
  • Rolando fracture (type II):
    
    • Comminuted Y- or T-shaped intra-articular fracture of the base of the 1st metacarpal.
      
    • Similar to a comminuted Bennett, these can be much more complex with multiple comminuted fractures.
      
  • Type III fractures
    
    • Extra-articular metacarpal fractures. Tend to be transverse or less commonly oblique.
      

• Falls, hyperflexion, hyperextension
  
• Motor vehicle accidents
  
• Sports, especially downhill or alpine skiing
  
• Basketball
  
• Baseball
  
• Football
  
• Rugby
  

DIAGNOSIS

• Pain, swelling, and deformity of the thumb
  
• Exam should include the thenar eminence for pain or deformity.
  
• The thumb may be rotated distal to the fracture site.
  
• The base of the thumb may appear radially deviated relative to the rest of the hand in the resting position.
  
• Occasionally, there may be damage to the thumb digital nerves.