What are the reasons for spleen issues and how are they treated?

Spleen Issues: Causes and Treatment

Spleen disorders are primarily caused by trauma, hematological conditions, and systemic diseases, and should be treated with appropriate vaccinations, antibiotic prophylaxis, and in some cases splenectomy to prevent life-threatening infections and complications. 1

Causes of Spleen Issues

Anatomical/Structural Issues

• Surgical removal (splenectomy) is performed for severe splenic trauma, splenic cysts, or as part of tumor resection procedures 1
• Partial splenectomy or autotransplantation may preserve some splenic function but still requires preventive measures similar to complete splenectomy 1

Functional Hyposplenism

• Hematological disorders including sickle cell anemia (HbSS, HbSC), thalassemia major, and essential thrombocythaemia can cause splenic dysfunction 1, 2
• Lymphoproliferative diseases often lead to functional hyposplenism 1, 3
• Autoimmune disorders including celiac disease, inflammatory bowel disease, dermatitis herpetiformis, autoimmune atrophic gastritis, autoimmune enteropathy, and autoimmune liver diseases can cause splenic hypofunction 1, 4
• Hyposplenism can be detected on blood film by the presence of red cells containing Heinz and Howell-Jolly bodies 1, 3

Other Causes of Splenomegaly

• Portal hypertension from liver diseases 5
• Infections (various types) 5, 6
• Malignancies including leukemias and lymphomas 5
• Collagen vascular diseases or Felty's syndrome 5
• Chronic hemolytic syndromes 5, 2
• Infiltrative diseases such as Gaucher's disease 5

Treatment and Management

Vaccination Protocol

• Pneumococcal vaccination is essential as the polyvalent vaccine contains 23 most prevalent serotypes and is >90% effective in healthy adults under 55 1
• Meningococcal vaccination should be administered to all asplenic patients 1
• Haemophilus influenzae type b (Hib) vaccination is critical, particularly for children 1, 6
• Influenza vaccination is recommended yearly to reduce risk of secondary bacterial infections 1
• Timing of vaccination should be at least two weeks before elective splenectomy to ensure optimal antibody response 1
• Reimmunization should be considered:
  • After two years in high-risk patients, especially children under 2 1
  • Every 5-10 years for asplenic patients 1

Antibiotic Prophylaxis

• Lifelong prophylactic antibiotics should be offered in all cases, especially in the first two years after splenectomy 1
• Phenoxymethylpenicillin has been used effectively for years, particularly in children with sickle cell anemia 1
• Erythromycin should be offered to patients allergic to penicillin 1
• Emergency antibiotics - patients should keep a supply of amoxycillin at home to use immediately if symptoms of infection develop 1

Special Considerations

• Children under 5 have a much higher infection rate (>10%) compared to adults (<1%) 1
• Duration of risk is lifelong, with cases of fulminating infection reported more than 20 years after splenectomy 1
• Animal bites require adequate antibiotic coverage (co-amoxiclav or erythromycin) due to risk of C. canimorsus infection 1
• Tick bites pose risk of babesiosis, particularly in those with animal contact 1
• Travel precautions should be taken, with immediate medical attention sought for any feverish illness 1

Monitoring and Education

• Patient education is crucial - information leaflets and patient cards about splenectomy should be provided 1, 6
• General practitioner notification of splenectomy and vaccinations given is important to avoid potential reactions 1
• Early recognition of hyposplenism in high-risk patients through screening is warranted 6
• Monitoring for pitted red cells can be used as an indicator of splenic function (normal upper limit 4%) 4

Pitfalls and Caveats

• Antibiotic prophylaxis limitations - phenoxymethylpenicillin does not cover H. influenzae, and amoxycillin may not reliably cover it either 1
• Vaccination timing - in young children with reduced efficacy, it may be better to rely initially on prophylactic antibiotics and immunize after the second birthday 1
• Overwhelming post-splenectomy infection remains a significant risk despite preventive measures 1, 6
• Splenomegaly with hyposplenism can coexist, making clinical assessment challenging 2, 3