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Hypersplenism

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  • Splenectomized patients should receive immunization to pneumococcus, meningococcus, Haemophilus influenzae, and influenza at least 14 days prior to splenectomy (2)[A].

  • If this cannot be done (i.e., in cases of emergent splenectomy), wait at least 14 days postsplenectomy to immunize.

    • Pneumococcal vaccine

      • Pneumococcal polyvalent-23 vaccine (PPSV23) for use in adults and fully immunized children ≥2 years of age

      • Pneumococcal polyvalent-13 vaccine (PCV13) for infants and young children ≥2 months of age as part of routine immunization schedule (3)[A]

      • PCV13 for children >2 years of age, adolescents and adults in addition to PPSV23; refer to CDC for timing of administration

      • Current guidelines recommend single revaccination of PPSV23 5 years after the initial dose and again at age of ≥65 years, at least 5 years after the previous dose.

    • H. influenzae vaccine

      • All unvaccinated individuals ≥ 5 years of age should be given 1 dose of H. influenzae type B (Hib) conjugate vaccine.

      • Children <5 years old should also be vaccinated. Refer to CDC for timing.

      • Vaccinated individuals can also be given additional dose of vaccine (4)[A].

    • Meningococcal vaccine (5)[A]

      • Meningococcal conjugate vaccine (MCV4) for use in patients between 2 and 55 years

      • Meningococcal polysaccharide vaccine (MPSV4) for use in patients >55 years of age

      • Revaccination is recommended every 5 years.

    • Influenza vaccine should be administered yearly based on prevalent circulating strains. Although patients are not at higher risk from influenza itself, infection with influenza may place patients at higher risk for secondary bacterial infections.

  • Radiofrequency ablation (RFA) is becoming more available and can be successful at preventing recurrence of hypersplenism. It is not currently known whether there are differences between RFA and splenectomy in terms of postprocedure infectious risks. Other alternatives to splenectomy include total and partial splenic embolization and shunting, although these techniques are evolving and additional studies are needed to evaluate efficacy and morbidity as compared to splenectomy.

 

INPATIENT CONSIDERATIONS


Admission Criteria/Initial Stabilization
  • Hypersplenism alone generally does not warrant admission. However, all patients should be monitored closely for complications of the resulting cytopenias, including bleeding and infection, as well as complications of splenomegaly, including increased risk of splenic rupture. In some patients, the large spleen compresses the stomach and prevents adequate oral intake.
  • Splenectomized patients are at increased risk of infection and postsplenectomy sepsis, especially with Streptococcus pneumoniae. Fevers, chills, or pain concerning for underlying infection warrant immediate attention as clinical decompensation can occur within hours. Empiric broad-spectrum antibiotics should not be delayed while evaluation is ongoing. Common empiric regimens include the following:
    • Ceftriaxone: 2g IV q24h and vancomycin 1 g IV q12h
    • Levofloxacin: 750 mg IV q24h and vancomycin 1 g IV q12h in β-lactam-allergic patients

ONGOING CARE


  • Adult patients who are splenectomized should be advised to monitor closely for fever or rigors at home, which may be an early sign of bacteremia. They should be instructed to begin antibiotics immediately prior to proceeding to a medical facility for evaluation. Early antibiotics have been shown to reduce the mortality from overwhelming postsplenectomy sepsis.
  • Controlled trials have not been performed, but some regimens include the following:
    • Amoxicillin-clavulanate: 875 mg PO twice daily
    • Cefuroxime axetil: 500 mg PO twice daily
  • Patients allergic to β-lactam antibiotics can be given an extended-spectrum fluoroquinolone such as levofloxacin 750 mg PO or moxifloxacin 400 mg PO daily.
  • In children with splenectomy, daily antibiotic prophylaxis for overwhelming postsplenectomy sepsis with penicillin VK or amoxicillin is recommended until age 5 or at least 3 years after splenectomy:
    • Age 2 months to 5 years: 125 mg PO BID
    • >5 years old: 250 mg PO BID

PATIENT EDUCATION


Patients who are splenectomized should be counseled extensively about the risk of overwhelming postsplenectomy sepsis and the need to obtain prompt medical evaluation in the event of fevers, chills, or any other concerning symptoms.  

REFERENCES


11 Bai  YN, Jiang  H, Prasoon  P. A meta-analysis of perioperative outcomes of laparoscopic splenectomy for hematological disorders. World J Surg.  2012;36(10):2349-2358.22 Advisory Committee on Immunization Practices. Recommended adult immunization schedule: United States, 2012. Ann Intern Med.  2012;156(3):211-217.33 American Academy of Pediatrics. Children with asplenia or functional asplenia. In: Pickering  LK, Baker  CJ, Kimberlin  DW, et al, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009:72.44 American Academy of Pediatrics Committee on Infectious Diseases. Haemophilus influenzae type b conjugate vaccines: recommendations for immunization with recently and previously licensed vaccines. Pediatrics.  1993;92(3):480-488.55 Centers for Disease Control and Prevention. Updated recommendations for use of meningococcal conjugate vaccines-Advisory Committee on Immunization Practices (ACIP), 2010. MMWR Morb Mortal Wkly Rep.  2011;60(3):72-76.

ADDITIONAL READING


  • Abdella  HM, Abd-El-Moez  AT, Abu El-Maaty  ME, et al. Role of partial splenic arterial embolization for hypersplenism in patients with liver cirrhosis and thrombocytopenia. Indian J Gastroenterol.  2010;29(2):59-61.
  • Di Sabatino  A, Carsetti  R, Corazza  GR. Post-splenectomy and hyposplenic states. Lancet.  2011;378(9785):86-97.
  • Feng  K, Ma  K, Liu  Q, et al. Randomized clinical trial of splenic radiofrequency ablation versus splenectomy for severe hypersplenism. Br J Surg.  2011;98(3):354-361.
  • Iriyama  N, Horikoshi  A, Hatta  Y, et al. Localized, splenic, diffuse large B-cell lymphoma presenting with hypersplenism: risk and benefit of splenectomy. Intern Med.  2010;49(11):1027-1030.
  • Jandl  JH, Aster  RH, Forkner  CE, et al. Splenic pooling and the pathophysiology of hypersplenism. Trans Am Clin Climatol Assoc.  1967;78:9-27.
  • Kapoor  P, Singh  E, Radhakrishnan  P, et al. Splenectomy in plasma cell dyscrasias: a review of the clinical practice. Am J Hematol.  2006;81(12):946-954.
  • Mourtzoukou  EG, Pappas  G, Peppas  G, et al. Vaccination of asplenic or hyposplenic adults. Br J Surg.  2008;95(3):273-280.
  • Shatz  DV, Schinsky  MF, Pais  LB, et al. Immune responses of splenectomized trauma patients to the 23-valent pneumococcal polysaccharide vaccine at 1 versus 7 versus 14 days after splenectomy. J Trauma.  1998;44(5):760-765.

SEE ALSO


Anemia, Autoimmune Hemolytic; Malaria; Polycythemia Vera; Tuberculosis  

CODES


ICD10


  • D73.1 Hypersplenism

ICD9


  • 289.4 Hypersplenism

SNOMED


  • 58381000 Hypersplenism (disorder)
  • 420749009 Hypersplenism associated with acquired immunodeficiency syndrome (disorder)

CLINICAL PEARLS


  • Splenectomy is not necessary to make the diagnosis.
  • Avoid splenectomy in patients unless absolutely necessary. Splenectomized patients are at lifelong risk for overwhelming postsplenectomy infection and sepsis.
  • If splenectomy is to be performed, give immunization for pneumococcus, meningococcus, Haemophilus, and influenza at least 14 days prior to surgery. Otherwise, wait until the 14th postop day to immunize.
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