What is the treatment for a 22-year-old female patient diagnosed with hereditary spherocytosis?

Treatment of Hereditary Spherocytosis

Splenectomy is the definitive treatment for hereditary spherocytosis, with vaccination against encapsulated organisms (pneumococcal, Haemophilus influenzae type b, and meningococcal) required at least 2 weeks before surgery, followed by lifelong antibiotic prophylaxis with phenoxymethylpenicillin. 1, 2

Indications for Splenectomy

Splenectomy should be performed in patients with:

• Moderate to severe hemolysis with hemoglobin levels below 80 g/L 3
• Transfusion-dependent anemia unresponsive to iron supplementation 3
• Symptomatic splenomegaly 3
• Recurrent hemolytic or aplastic crises 4
• Symptomatic cholelithiasis (perform concomitant cholecystectomy) 5, 3

Timing of Surgery

Delay splenectomy until after age 5-6 years when possible, as children under 5 years have the highest risk of overwhelming post-splenectomy infection (OPSI). 2, 4 The risk of OPSI is 0.5-2% with mortality rates of 30-70%, and most deaths occur within the first 24 hours of symptom onset. 2

Preoperative Requirements

Vaccination Protocol

• Complete all vaccinations at least 2 weeks before elective splenectomy to ensure optimal antibody response 1, 2
• 23-valent polysaccharide pneumococcal vaccine (>90% effective in healthy children) 2
• Haemophilus influenzae type B vaccine 2
• Meningococcal vaccine 2
• Annual influenza vaccine to reduce risk of secondary bacterial infection 2

Laboratory Assessment

• Complete blood counts, reticulocyte count, renal function tests, liver function tests, and bilirubin 2
• Blood typing and immune-hematological screening for possible erythrocyte antibodies 2
• Verify patient is at optimal baseline before proceeding 2

Contraindications

Active infection or acute hemolytic crisis are absolute contraindications for any elective, non-urgent splenectomy. 2

Surgical Approach

Laparoscopic splenectomy is the preferred approach when appropriately trained surgeons and suitable equipment are available. 5, 3 Total splenectomy remains the standard treatment in moderate and severe forms, though partial splenectomy may be considered to retain splenic immunologic function while decreasing hemolysis. 6

Perform concomitant cholecystectomy if gallstones are present, even if asymptomatic, as the incidence of cholelithiasis increases with age due to chronic hemolysis. 5, 3, 4

Postoperative Management

Immediate Care

• Maintain aggressive hydration, oxygenation, thermoregulation, and pain management to prevent vaso-occlusive complications 2
• Continue intravenous hydration until oral intake is fully re-established 2
• Provide supplemental oxygen to maintain transcutaneous saturation >95% 2
• Avoid hypothermia (do not use ice packs for pain management) 2
• Implement multimodal analgesia and encourage early mobilization 2

Vaccination if Missed Preoperatively

If vaccines were not administered preoperatively, vaccinate as soon as the patient is stable postoperatively, ideally within 14 days of surgery. 2 The antibody response is suboptimal if given before 14 days post-splenectomy, but vaccination before discharge is preferable to missing it entirely if follow-up compliance is uncertain. 2

Lifelong Infection Prevention

Antibiotic Prophylaxis

• Lifelong prophylactic phenoxymethylpenicillin (oral penicillin V) with particular emphasis on the first two years after splenectomy when risk is highest 2
• Erythromycin for patients allergic to penicillin 2
• Patients should keep a supply of amoxicillin at home to use immediately if symptoms of infection develop 2

Reimmunization Strategy

• Consider reimmunization after 2 years in children who remain at risk 2
• Long-term reimmunization every 5-10 years for asplenic patients 2

Special Precautions

• Animal bites require immediate antibiotic coverage with a 5-day course of co-amoxiclav (or erythromycin in allergic patients) due to susceptibility to Capnocytophaga canimorsus infection 2
• Additional prophylaxis for travel to endemic areas for histoplasmosis, babesiosis, and malaria 2

Patient Education

The risk of overwhelming infection is lifelong, with cases of fulminating infection reported more than 20 years after splenectomy. 2 Patients and families must understand that while most infections occur within the first two years, up to one-third may manifest at least five years later. 2

Provide patients with:

• Information leaflet and patient card about splenectomy for emergency situations 2
• Notification to general practitioner of the splenectomy and vaccinations given 2

Expected Outcomes

Splenectomy alleviates hemolysis and achieves clinical cure of anemia for most patients, though spherocytosis persists. 4 Patients with recessively inherited spherocytosis are exceptions—they benefit significantly from splenectomy but their anemia is not completely corrected. 4

Special Consideration for Elderly Patients

For patients diagnosed in their seventh to ninth decades with absent or mild clinical manifestations, careful observation with folic acid supplementation may be preferable to splenectomy, given the incidence of surgical complications in the elderly compared to the severity and incidence of disease complications. 7