Splenectomy for spherocytosis, URTI

Gabrielle Morris

25 year old patient for laparoscopic cholecystectomy and splenectomy due to hereditary spherocytosis.

Background

  • Hereditary spherocytosis
    • Pigment gallstones and biliary colic
    • Symptomatic splenomegaly
    • Hb drop from 132 to 107 over last 3 weeks
    • Recurrent severe haemolytic crises – Admission this year with nil precipitant, Hb drop to 40g/L
    • Pre-splenectomy vaccinations up to date
  • Anti-E antibodies (due to recurrent blood transfusion)
  • Smoker​

Issues:

  • Current URTI
    • COVID negative
    • Improving last 2 days, almost normal

Discussion

  • ​What is hereditary spherocytosis?
    • Autosomal dominant, abnormality of red cell membrane structural proteins
    • Classically diagnosed in children with haemolytic anaemia after Parvovirus infection, but may be asymptomatic
    • Variable severity.
    • Precipitated by infection
    • Splenic red cells sequestered in spleen, shortening T1/2 to as little as 10d.
    • Anaemia, splenomegaly, jaundice, increased reticulocyte count and spherocytes on peripheral blood smear.
    • Gallstones common, occurring in 50% of HS patients by 50yo. 
    • Treated symptomatically, with folic acid, transfusions, vaccinations (same as for splenectomy), cholecystectomy and splenectomy, as needed. 
    • Risk of overwhelming post-splenectomy infection (OPSI). Pneumococcal vaccination required preop. Prophylactic antibiotics used for at least 3 years, lifelong in some cases. Patients always carry emergency antibiotics with them, due to risk of rapid progression of sepsis. 
    • Splenectomy also carries a long-term thrombosis risk.
    • Spherocytes persist in the blood after splenectomy, but their lifespan is normalised. 
  • Should surgery proceed given current URTI?
    • Frequent URTIs in the community at present, may cause repeated delays to surgery.
    • ARISCAT scoring (assuming conversion to open abdominal procedure) suggests proceeding within 1 month of URTI in this​ patient would be associated with high risk (~44%) risk of POPC, dropping to moderate risk (~13%) thereafter.
    • Severity of haemolytic crisis this year is motivation to proceed.

Plan:

  • Proceed with surgery, as discussed with the treating surgeon.
  • Phone call to procedural anaesthetist