Q1. A 10 year old boy with T-ALL was admitted and his blood parameters at admission were:-
- CBC> Hb= 9 g/dl, Hct =28%, TLC= 102000/microl, PLT= 50000
- Clinical Chemistry > Creat 1.8, T Bil 1.2, D Bill 0.6, Uric Acid 12.3, K+ 5.8, Phosphate 6.8, Calcium 7.0, LDH 3000, SGOT/PT 65/62
Anti TLS measures including ample hydration, allopurinol and rasburicase, oral phosphate binders and anti hyperkelemia measures were given and his values were repeated after 6 hours which are as given below:-
- CBC> Hb= 6 g/dl, Hct =18%, TLC= 98000/microl, PLT= 48000
- Clinical Chemistry> Creat 1.6, T Bil 3.2, D Bill 0.9, Uric Acid 4.3, K+ 5.5, Phosphate 6.2, Calcium 7.8, LDH 13000, SGOT/PT 69/65
How will you explain this scenario ??
Answer:- Features are s/o Hemolysis
- Hemolytic anemia is likely to occur in G6PD-deficient patients due to their inability to break down hydrogen peroxide, which results from the oxidation of uric acid to allantoin.
- It is recommended that G6PD deficiency be ruled out prior to treatment with rasburicase, but this is not always feasible, as TLS is a dangerous condition that often progresses quickly if treatment is delayed.
- Thus, strict monitoring for signs and symptoms of hemolytic anemia is essential to provide appropriate supportive care if any adverse events occur.
- Currently the use of rasburicase in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency is contraindicated.