A 25 year old female with hereditary spherocytosis presents unwell with fevers, Rt sided chets pain and weakness.
She is pale looking
Vitals:
T 38.9 celcius
HR 120/min
BP 108/70
RR 22/min
O2 sats 98% RA
She has some crepitations in her rt lower zones of her lungs
The blood tests performed:
Hb 70 g/l
Platelets 206 (150-400)
WCC 7.7 (4-11)
Neutrophils 6.2 (4-7)
CRP 21
LDH 463 (<450 U/L)
Bilirubin 73 (<16 umol/L)
ALP 59 (20-105 U/L)
GGT 18 (<31 U/L)
ALT 18 (<31 U/L))
Questions:
1. Describe the abnormalities
2. Give a cause for her presentation
3. What are the clinical features of hereditary spherocytosis
4. What is the definitive treatment
5. What are the problems with this treatment