Do patients with hereditary spherocytosis require splenectomy?

Management of Hereditary Spherocytosis: Splenectomy Considerations

Splenectomy is not routinely required for all patients with hereditary spherocytosis and should be reserved only for those with severe disease manifestations including significant anemia, recurrent hemolytic crises, or growth failure.

Indications for Splenectomy in Hereditary Spherocytosis

Splenectomy should be considered in the following specific situations:

• Severe anemia requiring regular transfusions
• Recurrent hemolytic or aplastic crises
• Growth failure or skeletal changes due to over-robust erythropoiesis
• Symptomatic splenomegaly causing discomfort
• Gallstones due to chronic hemolysis

Disease Severity Assessment

Disease severity should be evaluated using:

• Hemoglobin concentration
• Reticulocyte count (potentially more reliable than hemoglobin for determining severity) 1
• Bilirubin levels
• Presence of splenomegaly
• Impact on quality of life and daily activities

Risks of Splenectomy

Splenectomy carries significant risks that must be weighed against potential benefits:

• Lifelong risk of overwhelming bacterial infections (not limited to pneumococcus, meningococcus, and H. influenzae) 2
• Increased risk of thromboembolism (3-4 fold higher risk of pulmonary embolism) 3
• Potential increased risk of malignancy (4.7-fold higher risk of non-Hodgkin lymphoma) 3
• Perioperative complications (10% with laparoscopic approach) 3
• Mortality risk (0.2% with laparoscopic approach, 1.0% with open approach) 3

Alternative Approaches

For patients with moderate disease who may benefit from reduced splenic hemolysis but want to maintain some splenic function:

• Partial splenectomy - preserves immunologic function while decreasing hemolysis rate 4
• Partial splenic embolization - shown to significantly increase hemoglobin levels and decrease splenic size 5

Post-Splenectomy Management

If splenectomy is performed:

• Vaccinate against encapsulated organisms at least 4 weeks before (preferably) or 2 weeks after splenectomy
  • Pneumococcal vaccine
  • Meningococcal vaccine
  • H. influenzae type b vaccine
• Provide patient education about risk of post-splenectomy infection
• Consider home supply of antibiotics for febrile illness
• Provide medical alert card/bracelet indicating asplenic status
• Monitor for thrombocytosis post-operatively

Decision Algorithm

1. Assess disease severity:

   • Severe: Hb <8 g/dL, reticulocyte count >10%, transfusion dependence
   • Moderate: Hb 8-11 g/dL, reticulocyte count 6-10%
   • Mild: Hb >11 g/dL, reticulocyte count <6%

2. For severe disease:

   • Consider total splenectomy if patient is >6 years old
   • Consider partial splenectomy if patient is <6 years old

3. For moderate disease:

   • Consider partial splenectomy or partial splenic embolization
   • Monitor and reassess if symptomatic

4. For mild disease:

   • Observe and monitor
   • Consider intervention only if complications develop (gallstones, growth failure)

Conclusion

The evidence suggests that a conservative approach to splenectomy in hereditary spherocytosis is warranted due to the lifelong risks associated with the procedure. The decision should be based primarily on disease severity and specific complications rather than performing splenectomy as a routine procedure for all patients with hereditary spherocytosis.