Hematologic / oncologic disorders - aa

Question 1

~progressive pancytopenia & macrocytosis
~purpura, petechiae, bleeding
~cafe au lait spots, hypopigmentation
~short stature & delicate features
~skeletal malformations e.g. missing thumb & radius
~may be cured by successful bone marrow transplant

Answer 1

aplastic anemia

Question 2

~hypochromic microcytic anemia
~symptoms range from asymptomatic to mild to moderate to fetal demise
~transfusions may be required

Answer 2

thalassemia

Question 3

hydrops fetalis

Answer 3

the worst kind of alpha thalassemia - all four alpha-globin genes are deleted; no normal hemoglobin is produced, so fetus is stillborn

Question 4

~recurrent episodes of musculoskeletal and/or abdominal pain
~pallor, jaundice, predisposition to gallstones during childhood
~poorly healing ulcers over lower tibia
~dactylitis in up to 50% of kids before age 3
~diagnosis confirmed with hemoglobin electrophoresis
~treatment: daily prophylactic penicillin from 2 months through 5 years

Answer 4

sickle cell anemia

Question 5

~pallor, fatigue
~nutritional deficiency
~microcytic hypochromic anemia

Answer 5

iron deficiency anemia

Question 6

The most common red cell enzyme defect that causes hemolytic anemia.

Answer 6

G6PD deficiency

Question 7

~significant hyperbilirubinemia in infants
~older kids asymptomatic and normal-appearing between episodes of hemolysis
~bite cells, spherocytes, Heinz bodies

Answer 7

G6PD deficiency

Question 8

~Otherwise healthy child
~Markedly decreased platelet count
~Petechiae, ecchymoses
~Most common bleeding disorder of childhood
~2-5 yo, often following viral infection
~avoid NSAIDs
~usually recover spontaneously

Answer 8

idiopathic thrombocytopenic purpura

Question 9

~bruising, hemarthrosis
~reduced factor VIII activity
~prolonged aPTT
~treatment: exogenous factor VIII; desmopressin

Answer 9

hemophilia

Question 10

The most common childhood brain tumor.

Answer 10

astrocytoma

Question 11

Classic brain tumor triad:

Answer 11

morning headache
vomiting
papilledema

Question 12

Infratentorial brain tumors in what age group?

Supratentorial brain tumors in what age group?

Answer 12

infratentorial: less than 6
supratentorial: greater than 8

Question 13

Hx of a sports injury that doesn’t get better; bone pain; tall kids; sunburst pattern on xr

Answer 13

osteosarcoma

Question 14

Involves diaphyses of long bones; pain at tumor site; moth-eaten lytic lesion on xr

Answer 14

Ewing sarcoma

Question 15

Abdominal mass, smooth firm, well-demarcated. Overall cure rate of 90%

Answer 15

Wilms tumor