II. Causes: Hyposplenism or Asplenism

  1. Common Surgical Causes
    1. Splenectomy
    2. Partial Splenectomy
  2. Common Medical Causes
    1. Celiac Disease
    2. Cirrhosis
    3. Sickle Cell Anemia
    4. Vasculitis
    5. Systemic Lupus Erythematosus
    6. Bone Marrow Transplantation
    7. Splenic irradiation in Hodgkin's Disease
  3. Other Causes of Hyposplenism
    1. Cyanotic heart disease
    2. Ulcerative Colitis
    3. Whipple's Disease
    4. Chronic active hepatitis
    5. Acute Alcohol Abuse
    6. Hemoglobinopathy
    7. Primary Thrombocythemia
    8. Fanconi's Syndrome
    9. Malignant histiocytosis
    10. Rheumatoid Arthritis
    11. Grave's Disease
    12. Sarcoidosis
    13. Splenic arterial or venous Occlusion
    14. HIV Infection
    15. Total Parenteral Nutrition
    16. High dose Corticosteroids

III. Pathophysiology: High Risk Organisms

  1. Encapsulated Bacteria
    1. Streptococcus Pneumoniae
    2. HaemophilusInfluenzae
    3. Neisseria Meningitidis
  2. Gram Negative Bacteria
    1. Capnocytophaga canimorsus (Dog Bites)
  3. Intraerythrocytic Parasites
    1. Plasmodia falciparum (Malaria)
    2. Babesia microti (Babesiosis)
  4. Less common organisms
    1. Group B Streptococcus
    2. Enterococcus
    3. Bacteroides
    4. Salmonella
    5. Bartonella
    6. Plesiomonas shigelloides
    7. Eubacterium plauti
    8. Pseudomonas pseudomallei

IV. Diagnostics to identify Hyposplenism

  1. Step 1: History suggests possible Hyposplenism
    1. Known post-Splenectomy: See Management below
    2. Possible Hyposplenism: Go to Step 2
  2. Step 2: Peripheral Smear shows Howell-Jolly Bodies
  3. Step 3: Abdominal Ultrasound if smear positive
    1. Spleen absent
      1. See Management below
    2. Spleen present
      1. Radionuclide liver-Spleen scan
  4. Step 4: Radionuclide liver-Spleen scan
    1. Normal Uptake
      1. Normal splenic function
    2. Decreased or absent splenic uptake
      1. See management below

V. Management: Prevention

  1. Initial Immunizations
    1. Timing
      1. Preferred >14 days before elective splenectomy
      2. Otherwise administer on diagnosis
    2. Immunizations
      1. Pneumococcal Vaccine
        1. PPV-23 (Pneumovax) for adults and children who completed PCPV-7
        2. PCPV-7 (Prevnar) for children and adults who Overwhelming Postsplenectomy Infection
      2. Meningococcal Vaccine (Menactra)
        1. Two dose series (with 2 month interval) and
        2. Future boosters every 5 years
      3. Haemophilus B Conjugate Vaccine
        1. Primary Series in children (four doses)
        2. No guidelines on booster Immunization in Asplenic adults
  2. Repeat Pneumococcal Vaccine (PPV-23) after age 10 years
    1. Every 5 years for most Asplenic patients
    2. Every 3 years for early waning Antibody titers
      1. Hypogammaglobulinemia
      2. Sickle Cell Anemia
      3. Nephrotic Syndrome
      4. Chronic Renal Failure
  3. Documentation and Education
    1. Medic-Alert Bracelet
    2. Update medical record
      1. Vaccinations
      2. Asplenism
    3. Patient educated on infectious risks of Asplenism
      1. Immediate evaluation for febrile illness
      2. Notify dentists and doctors of Asplenic state
      3. Exposure risks
        1. Travel (Malaria)
        2. Tick Bites (Ehrlichia and Babesiosis)
        3. Dog Bite

VI. Management: Antibiotics for prophylaxis or as Stand-by at first signs of infection

  1. Antibiotics: Daily prophylaxis
    1. Amoxicillin
    2. Penicillin
    3. Trimethoprim-Sulfamethoxazole (Bactrim)
  2. Antibiotics: Stand-by Antibiotics
    1. Amoxicillin-Clavulanate
    2. Cefuroxime (Zinacef)
    3. Levofloxacin (Levaquin) - in adults
  3. Protocols
    1. Children with Asplenism or Hyposplenism
      1. Daily Prophylaxis
      2. Duration controversial (options below)
        1. First 2-5 years after splenectomy
        2. Continued until age 21 years
        3. Continue daily prophylaxis if history of Overwhelming Postsplenectomy Infection
    2. Adults with Asplenism or Hyposplenism
      1. Continue daily prophylaxis if history of Overwhelming Postsplenectomy Infection
      2. Standby antibiotics taken at first signs infection (fever)
        1. Augmentin or
        2. Levofloxacin
      3. Immediate medical evaluation mandatory
        1. Well-appearing: Obtain cultures and continue antibiotics for 7-10 days
          1. Consider 24 hour follow-up
        2. Suspect Sepsis: Admit immediately and start IV antibiotics
          1. See Overwhelming Postsplenectomy Infection

VII. Management: Acute Infection

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Ontology: Hyposplenism (C0272404)

Concepts Disease or Syndrome (T047)
ICD10 D73.0
SnomedCT 23761004
English hyposplenism, hyposplenism (diagnosis), Hyposplenism, Hyposplenism (disorder)
German Hyposplenismus
Korean 지라기능감퇴증
Dutch Hyposplenie
Spanish hipoesplenismo (trastorno), hipoesplenismo

Ontology: Functional asplenia (C0272405)

Concepts Disease or Syndrome (T047)
SnomedCT 38096003
English Functional asplenia, Functional asplenia (disorder)
Spanish asplenia funcional (trastorno), asplenia funcional

Ontology: Post-splenectomy disorder (C0398662)

Concepts Disease or Syndrome (T047)
SnomedCT 234511009
English Post-splenectomy disorder, Post-splenectomy disorder (disorder)
Spanish trastorno posesplenectomía (trastorno), trastorno posesplenectomía

Ontology: Congenital absence of spleen (C0600031)

Concepts Congenital Abnormality (T019)
ICD10 Q89.01
SnomedCT 38096003, 205732008, 702624008, 93030006, 17604001
English Asplenia, asplenia, asplenia (diagnosis), Asplenia (congenital), Congenital absence of spleen, Absent spleen, (Absent spleen) or (asplenia) (disorder), (Absent spleen) or (asplenia), Aplasia of spleen (disorder), Splenic aplasia, Aplasia of spleen, Splenic agenesis, Congenital asplenia, Congenital absence of spleen (disorder), agenesis; spleen, spleen; agenesis, Congenital absent spleen
Italian Asplenia
Japanese 無脾, ムヒ
Czech Asplenie
Hungarian Asplenia
Dutch agenesie; milt, milt; agenesie, asplenie
Spanish agenesia esplénica, aplasia esplénica, asplenia congénita, ausencia congénita del bazo (trastorno), ausencia congénita del bazo, Asplenia
Portuguese Asplenia
French Asplénie
German Asplenie