Updated: 3/27/2020


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  • A 13-year-old girl presents to her primary care physician for follow-up care of her recently diagnosed hereditary spherocytosis. She reports increasing fatigue and shortness of breath. She required a blood transfusion approximately 4 months prior to presentation. She has not received the PCV13 vaccine but has received the PPSV23 vaccine on year ago. Physical examination shows pallid conjunctiva and organomegaly in the left upper quadrant. Laboratory testing is significant for an elevated reticulocyte count and serum bilirubin. A decision has been made among the patient, her family, and physician to undergo an elective splenectomy. She is administred the PCV13 vaccine 14-days before her operation.
  • Clinical definition
    • absence of normal spleen function that can be secondary to
      • surgical splenic removal
      • congenital absence or abnormally reduced spleen
      • splenic infarction such as in
        • sickle cell disease and thrombosis of the splenic artery
      • splenic infiltration such as in
        • sarcoidosis, malignancy, and amyloidosis
  • Under normal conditions
    • the spleen is involved in
      • filtering the blood through a series of capillaries, which results in
        • the removal of rigid erythrocytes
        • ingestion of circulating bacteria
    • the spleen is a lymphoid organ that contains
      • B-lymphocytes which is involved in
        • producing opsonizing antibodies against foreign materials which is vital for
          • eliminated encapsulated organisms
  • Patients with absent splenic function are therefore
    • at risk of infection by encapsulated organisms such as
      • Streptococcus pneumoniae (most important) 
      • Haemophilus influenzae
      • Neisseria meningitidis
    • other infectious agents include
      • Capnocytophaga canimorsus
        • patients are at risk when exposed to dogs
      • Babesia
  • Physical exam
    • in cases of sepsis
      • toxic appearing
      • tachycardia
      • evolving hypotension
      • altered sensorium
  • Labs
    • blood smear will show
      • Howell-Jolley bodies
      • target cells
    • complete blood cell count (CBC) can show
      • leukocytosis
      • thrombocytosis
  • Conservative
    • immunization
      • indication
        • should be administered at least two weeks before a scheduled splenectomy
        • should be administered two weeks after splenectomy in patients who
          • were not able to receive pre-operative immunization
      • modality
        • PCV13 and PPSV23 is recommended for both adults and children
  • Medical
    • antibiotic prophylaxis  
      • indication
        • this is the mainstay and initial treatment for infection prophylaxis in asplenic patients
        • children should receive daily antibiotic prophylaxis (amoxicillin or penicillin VK) until
          • the age of five and
            • at least one year post-splenectomy
        • must be taken when fever or rigors is present in asplenic patients since this is
          • can be a warning sign for fulminant bacteremia
          • antibiotics include
            • amoxicillin-clavulanate or cefuroxime in children
            • fluoroquinolone (e.g., levofloxacin) in adults
  • Sepsis due to
    • infection by encapsulated organisms

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