Hematology/Oncology

Question 1

What are three risk factors for more frequent vaso-occlusive crises in children with sickle cell?

Answer 1

1. Severe genotype (HgbSS or HgbSBo)
2. Increasing age (peaks in mid-20s)
3. High baseline hemoglobin level
   NB: High baseline fetal hemoglobin level is protective

Question 2

What is the role of RBC transfusions in vaso-occlusive crises in children with sickle cell disease?

Answer 2

No role.
Main stay of treatment for VOC is:
1. Opioids
2. Anti-inflammatory
3. IV fluids

Question 3

What does Hydroxyurea do for sickle cell disease patients?

Answer 3

Hydroxyurea increases Hemoglobin F expression to decrease polymerization of Hemoglobin SS

Question 4

Which sickle cell genotypes get hydroxyurea?

Answer 4

Hydroxyurea is offered to all children with SS or SBo disease

Question 5

What does the transcranial doppler (TCD) Ultrasound predict in patients with sickle cell disease?

Answer 5

The TCD predicts the risk of acute stroke in SS and SBo patients. An abnormal TCD examination represents a 40% risk of stroke in the subsequent 3 years
Annual TCD screening is standard of care

Question 6

What will you see on your physical exam and laboratory findings when you suspect a sickle cell patient with splenic sequestration?

Answer 6

1. Splenomegaly
2. Low hemoglobin from baseline
3. Thrombocytopenia

Question 7

What is the current practice for pneumococcal immunization and prophylactic penicillin use for sickle cell disease patients?

Answer 7

1. Provide daily penicillin prophylaxis beginning at before 2 months of age
2. Provide the PCV13 series that is recommended for all children as per the immunization guidelines
3. Provide 2 doses of pneumococcal polysaccharide vaccine (first dose at 2 years of age)

It is debatable whether daily penicillin prophylaxis could be discontinued at 5 years of age