Second Level Questions

Question 1

A five-year-old boy presents to clinic with a red tongue, cracked lips, fever, and peeling hands. What treatment will you give him immediately?

Answer 1

IVIG and aspirin

This is Kawasaki Disease. Treatment with IVIG and aspirin is meant to prevent coronary aneurysms and therefore prevent MI.

Question 2

Marrow biopsy is hypercellular with micromegakaryocytes. Peripheral blood shows increased granulocytes. What is the mechanism of the first-line therapy for treating this condition?

Answer 2

Imatinib is a tyrosine kinase inhibitor. It binds near the active site on BCR-ABL, preventing it from being able to phosphorylation other proteins.

This cancer is CML. Imatinib only works if the patient has the t(9;22) BCR-ABL translocation.

Question 3

A patient presents with pancytopenia but a hypercellular marrow. The patient has a 5q deletion. How does this disease tend to evolve, and with what prognosis?

Answer 3

This is a myelodysplastic disorder. It is characterized by low peripheral counts with dysplastic morphology (hyposegmented hypogranular neutrophils, abnormal RBCs, increased RBC MCV, weird platelets), and a hypercellular marrow. It has the tendency, especially with monosomy 7, trisomy 8, or 5q deletion, to evolve to AML with an increasing percentage of blasts. This kind of AML has a very poor prognosis.