Rosen & Barkin's 5-Minute Emergency Medicine Consult (388 page)

• Glucocorticoids (high dose over 2–3 wk):
  
  • Dexamethasone: 40 mg PO daily
    
  • Prednisone: 1–2 mg/kg/24 hr PO/day
    
  • Methylprednisolone: 1 g IV q8h (30 mg/kg/24 hr pediatric dose)
    
• IVIG: 1–2 g/kg IV × 1 dose and possibly repeated in 24 hr:
  
  • Used only for critical bleeding or when the need to acutely raise platelets is required such as emergency surgery
    
• Anti-D immunoglobulin: 50 μg/kg/24 hr IV:
  
  • Used only presplenectomy and in Rh
    ⁺
    patients
    

• Chronic long-term suppression and steroid bolus therapy
  
• Immunosuppressive agents:
  
  • Azathioprine
    
  • Cyclosporin
    
  • Mycophenolate
    
  • Chemotherapeutic agents:
    
  • Vinca alkaloids
    
  • Cyclophosphamide
    
  • Combinational chemotherapy
    
• Other:
  
  • Rituximab: Monoclonal antibody directed against B cell antigens
    
  • Danazol: Antiandrogen
    
• Experimental:
  
  • Other monoclonal antibodies directed at B cells
    
  • Stem cell transplants
    
  • Thrombopoietin and thrombopoietin-like agonists
    

FOLLOW-UP

• Life-threatening bleeding regardless of platelet count
  
• Any bleeding with platelet count <20 K
  
• Asymptomatic patient with platelet count <20 K with issues of noncompliance or poor follow-up
  

• Asymptomatic patients
  
• Patients with minor bleeding and platelets >30 K
  

Hematology referral is indicated in all cases (either outpatient or inpatient consultation)

• Before low platelet counts are evaluated, pseudothrombocytopenia should be excluded.
  
• Spontaneous bleeding usually does not occur until platelet counts are <10 K.
  

• British Committee for Standards in Haematology General Haematology Task Force. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy.
  Br J Haematol
  . 2003;120:574–596.
  
• Fogarty PF, Segal JB. The epidemiology of immune thrombocytopenic purpura.
  Curr Opin Hematol
  . 2007;14:515–519.
  
• Godeau B, Provan D, Bussel J. Immune thrombocytopenic pupura in adults.
  Curr Opin Hematol
  . 2007;14:535–556.
  
• Neunert C, Lim W, Crowther M, et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia.
  Blood
  . 2011;117(16):4190–4207.
  
• Schipperus M, Fijnheer R. New therapeutic options for immune thrombocytopenia.
  Neth J Med.
  2011;69(11):480–485.
  

See Also (Topic, Algorithm, Electronic Media Element)

HELLP Syndrome; Thrombotic Thrombocytopenic Purpura

CODES

287.31 Immune thrombocytopenic purpura

BASICS

• Immunization enhances or initiates resistance to infectious diseases.
  
• Protection from immunization occurs through several mechanisms:
  
  • Passive immunization: Administration of purified antibodies or passive transfer of maternal antibodies through the placenta/breast milk.
    
  • Active immunization: Stimulates immune system, producing IgM antibodies after 7–10 days followed by IgG antibodies, peaking between 2 and 6 wk.
    
• Oral and nasal vaccines induce mucosal secretory IgA antibodies while parenteral vaccines may not. Improper administration (route, dose, bad storage, etc.) may result in decreased immunity.
  

• Several types of vaccines are available:
  
  • Live attenuated (weakened) viruses (e.g., varicella [VZV]; measles, mumps, rubella [MMR]; rotavirus) replicate in the host and induce an immune response:
    
    • May cause serious infections in the immunocompromised.
      
  • Inactivated (or killed) vaccines (e.g., polio [IPV], hepatitis A [HepA], some influenza, pertussis) are safe in patients with compromised immune system.
    
  • Toxoid, subunit, or conjugate vaccines (e.g., diphtheria, tetanus,
    Haemophilus influenzae b
    [Hib], Human papilloma virus [HPV], Pneumococcus, Meningococcus) use antigenic portions of toxins, proteins, or carbohydrates from viruses or bacteria to induce immune response
    
  • Hepatitis B (HepB) vaccine uses recombinant DNA technology
    
• Several combination vaccines are also available but have an increased cost:
  
  • Pediarix (diphtheria and tetanus toxoids and acellular pertussis adsorbed [DTaP], HepB, and inactivated poliovirus vaccine [IPV] combined)
    
  • Comvax (HepB and Hib).
    
  • Pentacel (DTaP, IPV, and Hib).
    
  • Twinrix (HepA and HepB).
    
  • MMRV (MMR and varicella).
    

• The incidence of several life-threatening illnesses has been markedly reduced with widespread immunization use:
  
  • Polio caused by wild-type viruses has been eliminated from the Western Hemisphere.
    
  • Hib, diphtheria, and tetanus vaccines have nearly eliminated these invasive diseases among children in North America.
    
  • The incidence of measles, rubella, and varicella has also declined; sporadic in unimmunized communities and foreign travelers to US.
    
  • 7-valent and 13-valent conjugate pneumococcal vaccines (Prevnar 7 and Prevnar 13) have reduced invasive disease from 100–21 cases/100,000 for all types of pneumococcal infection and from 80–0.2/100,000 for vaccine serotypes (99% reduction).
    
  • Rotavirus is a live oral vaccine
    
  • Respiratory syncytial virus immune globulin given to high-risk patients
    
  • Global surveillance of influenza activity allows for annual production of vaccines against seasonal influenza. Inactivated vaccines are available for IM administration and live attenuated virus vaccines can be given via the nasal route.
    
• Immunization recommendations and schedules are based on epidemiology, individual risks for disease and exposure, as well as vaccine safety and efficacy:
  
  • Infants and young children are vaccinated against common childhood diseases, but some vaccines are not immunogenic (e.g., pneumococcal capsular polysaccharide antigen vaccine) or may be dangerous (e.g., MMR, VZV) in infants.
    
  • Pregnant women are recommended to receive Tdap and inactivated influenza, but should not receive other live virus vaccines.
    
  • Specific recommendations exist for other at-risk groups including international travelers, the elderly, health care workers, and immunocom-promised individuals.
    

DIAGNOSIS

• Concern for vaccine-associated adverse events: The most common events associated with vaccination are mild and include local reactions (pain, swelling, erythema) and/or fever (usually within 7–10 days):
  
  • Local reactions can occur with almost any injected vaccine, but are particularly common with tetanus, diphtheria, and pertussis (particularly after repeat doses), Hib (can cause sterile abscesses in young infants), VZV, HPV, and pneumococcus.
    
  • Fever may follow immunization with rotavirus (40–43%), conjugate pneumococcus (24–35%), HPV (10–13%), MMR or MMRV (more common in the latter), and meningococcal vaccine. Influenza vaccine may cause fever 6–24 hr after administration.
    
  • Rash may be seen as a rare side effect of VZV and MMRV, MMR may cause transient rash or fever 6–12 days after immunization.
    
  • Neurologic symptoms are rare but most commonly seen after DTP vaccines (fussiness, inconsolable crying, drowsiness, brief seizures without fever, hypotonic–hyporesponsive episodes, encephalopathy, and Guillain–Barré syndrome). Headache may follow vaccination with HepB or meningococcus; and MMR and MMRV are associated with febrile seizures in children and rare reports of encephalopathy. Guillain–Barré has also been linked to influenza vaccine in adults.
    
  • Vomiting and diarrhea are rare adverse effects of rotavirus vaccine in infants.
    
  • MMR has been associated with arthralgias in adult women, and rare hematologic adverse events such as thrombocytopenia.
    
  • Live attenuated influenza (nasal) has been associated with mild respiratory symptoms in adults and asthma exacerbations in children with a history of asthma.