What is the treatment for hereditary spherocytosis?

Treatment of Hereditary Spherocytosis

Splenectomy is the standard surgical treatment for moderate to severe forms of hereditary spherocytosis (HS), with partial splenectomy being a viable alternative to reduce long-term infection risks while still improving hematologic parameters. 1

Indications for Splenectomy

• Splenectomy is indicated in patients with:
  • Severe anemia 2
  • Recurrent hemolytic crises 2
  • Aplastic crises 2
  • Growth retardation or skeletal changes due to expanded bone marrow 1

Types of Splenectomy

Total Splenectomy

• Traditional approach that completely removes the spleen 1
• Most effective at resolving anemia and reducing transfusion requirements 2
• Associated with lifelong risk of potentially lethal infections 1
• Platelet counts increase significantly post-surgery but return to preoperative levels within a year 2

Partial/Near-Total Splenectomy

• Preserves 10-20% of splenic tissue to maintain immunologic function while decreasing hemolysis 3
• Comparable improvement in hematologic parameters to total splenectomy 2
• Reduces risk of overwhelming post-splenectomy infection 1
• Surgical technique considerations:
  • Branches of splenic arteries provide better blood supply than short gastric vessels 2
  • Near-total splenectomy (NTS) aims to conserve a remnant spleen of approximately 10 cm in size 3
  • Remnant spleen typically grows back to about 4.5 times its postoperative size 3

Preoperative Management

• Complete blood count with reticulocyte count to establish baseline 1
• Vaccination against encapsulated organisms:
  • Pneumococcal vaccine
  • Haemophilus influenzae type b vaccine
  • Meningococcal vaccine 4
• These should be administered at least 2 weeks before elective splenectomy 1

Postoperative Management

• Monitor for:

  • Platelet count (typically elevated for 6 months post-surgery) 2
  • Hemoglobin levels (should improve) 2
  • Bilirubin levels (typically decrease initially, then stabilize at levels lower than preoperative values) 2

• Antibiotic prophylaxis:

  • Recommended for children after splenectomy 5
  • Duration varies by institutional protocols, but typically continues through childhood 4

Outcomes

• Laboratory parameters typically improve in all patients:

  • Increased hemoglobin concentration 2, 3
  • Decreased bilirubin levels 2
  • Improved red blood cell count 2
  • Reduced or eliminated need for transfusions 3

• Recent evidence suggests that:

  • HS patients actually have a higher risk of infection before splenectomy than after (OR 6.6; 95% CI 3.0-14.2) 4
  • With proper vaccination and antibiotic prophylaxis, post-splenectomy infection risk is comparable to the general population 4
  • Splenectomy may reduce hospitalization for infections by alleviating HS complications 4

Special Considerations

• For patients with splenic sequestration:

  • Surgical splenectomy may be recommended after recovery from life-threatening or recurrent episodes 6
  • Care must be taken to avoid acute overtransfusion to a hemoglobin greater than 10 g/dL 6

• Laparoscopic approach:

  • Associated with shorter hospital stays and less postoperative pain 1
  • Similar operating time compared to open procedures 2
  • Feasible for both total and partial splenectomy 2

• Potential complications to monitor:

  • Secondary remnant necrosis (rare, reported in approximately 5% of partial splenectomy cases) 3
  • Excessive regeneration of residual parenchyma in some partial splenectomy techniques 3

In conclusion, the benefits of splenectomy in children with hereditary spherocytosis who have a significant disease burden typically outweigh the risks when appropriate preventive measures are taken 4.