How should hereditary spherocytosis be treated in adult patients?

Treatment of Hereditary Spherocytosis in Adults

Splenectomy is the definitive treatment for adults with hereditary spherocytosis who have symptomatic anemia, transfusion dependence, or complications such as cholelithiasis, and should be performed after appropriate vaccination against encapsulated organisms. 1, 2, 3

Pre-Splenectomy Vaccination Requirements

Before proceeding with elective splenectomy, vaccination against encapsulated organisms is mandatory and must be completed at least 2 weeks prior to surgery 1:

• Pneumococcal vaccine 1
• Haemophilus influenzae type b vaccine 1
• Meningococcal vaccine (based on general medical knowledge for asplenic patients)

Indications for Splenectomy

Splenectomy should be performed in adults with hereditary spherocytosis when any of the following are present 2, 3, 4:

• Symptomatic anemia unresponsive to supportive care (hemoglobin <80 g/L with symptoms) 2
• Transfusion dependence or increased transfusion requirements 2
• Symptomatic splenomegaly causing abdominal discomfort or risk of traumatic rupture 2, 3
• Cholelithiasis with symptoms requiring intervention 2, 3
• Recurrent hemolytic or aplastic crises 4

Surgical Approach

Total splenectomy remains the standard and most effective surgical treatment for moderate to severe hereditary spherocytosis in adults 2, 5, 3:

• Laparoscopic splenectomy is preferred when feasible, with lower complication rates compared to open surgery 2
• Open splenectomy is reserved for cases with massive splenomegaly or technical limitations 2
• Concurrent cholecystectomy should be performed if gallstones are present at the time of splenectomy 2, 3
• Identification and removal of accessory spleens is critical during surgery to prevent recurrent hemolysis 3

Partial Splenectomy Consideration

Partial (subtotal) splenectomy may be considered as an alternative to preserve some splenic immune function while reducing hemolysis 5:

• This approach aims to retain immunologic protection while decreasing hemolysis rates 5
• However, long-term outcomes remain less well-established compared to total splenectomy 5
• In adults with established disease requiring intervention, total splenectomy provides more definitive resolution 2, 3

Expected Outcomes After Splenectomy

Following total splenectomy 2, 3, 4:

• Spherocytosis persists on blood smear, but hemolysis is dramatically reduced or eliminated 3
• Clinical cure of anemia is achieved in most patients with dominantly inherited disease 3
• Transfusion requirements are eliminated in the vast majority of cases 2, 5
• Risk of future gallstone formation is substantially reduced 3
• Patients with recessively inherited spherocytosis may have persistent mild anemia despite significant improvement 3

Critical Pitfalls to Avoid

Do not perform splenectomy without completing the vaccination series at least 2 weeks beforehand—this exposes patients to life-threatening risk of overwhelming post-splenectomy sepsis from encapsulated organisms 1

Do not overlook accessory spleens during surgery—failure to remove accessory splenic tissue results in persistent hemolysis and treatment failure 3

Do not miss concurrent cholelithiasis—if gallstones are present at the time of splenectomy, perform cholecystectomy during the same operation to avoid a second procedure 2, 3

Avoid acute overtransfusion to hemoglobin >10 g/dL in patients with splenic sequestration, as this can precipitate complications 1

Non-Surgical Management

For asymptomatic or mildly symptomatic adults with well-compensated hemolysis 4:

• Observation without intervention is appropriate for patients with minimal anemia and no complications 4
• Folic acid supplementation (typically 1 mg daily) to support increased erythropoiesis
• Regular monitoring for development of complications (anemia progression, gallstones, hemolytic crises)
• Iron supplementation is not indicated unless true iron deficiency is documented, as these patients have chronic hemolysis with adequate iron stores

Long-Term Post-Splenectomy Surveillance

After splenectomy, patients require 3, 4:

• Lifelong awareness of infection risk from encapsulated organisms
• Prompt evaluation and empiric antibiotics for febrile illnesses
• Consideration of prophylactic antibiotics in high-risk situations (depending on individual risk assessment)
• Monitoring for rare thrombotic complications in the post-splenectomy state