Sickle Cell Disease Dec 2003

Acute Splenic Sequestration JP/IAS/KSC

Definition: -decrease in haemoglobin by at least 2g/dl

-Acutely enlarging spleen
-High reticulocyte count

Clinical Features: Consider differential diagnosis:

 Shock
 Pallor  Sepsis (patient usually febrile)- give
 Large, tender spleen cefotaxime and macrolide antibiotics
 Sickle Cell Disease (usually SS)
 Usually age 6 months – 5 years  Acute chest syndrome (respiratory distress)
 May have past history of sequestration
 Aplastic Crisis (onset less acute; no
abdominal pain)

Splenic Sequestration confirmed?:

 Call senior on call.
 Senior to inform haematologist on call
o (Dr K Charles or Dr Capildeo)

Resiscitation:
 Give high-flow oxygen and stabilize ABC’s
 Establish IV access
o Give fluids judiciously!! (Patients need BLOOD)
o Take bloods for CBC; retics; URGENT x-match,
O negative blood if emergency
 Top-up transfusion to steady state only up to Hb 8g/dl
 Monitor patient – ECG; SpO2; blood pressure in A&E
 Note: acute chest syndrome present in at least 20% of all
cases, thus proper chest examination and chest X ray in
all cases
 Admit to the ward when stable or consider ICU if
unstable after d/w seniors

Remember in appropriate follow-up:

 For discussion with paediatric surgery, haematologist, and
paediatrician re: long term follow up which may include:
1) chronic transfusion treatment
2) splenectomy
3) observation before definitive treatment decision is made