Heme/onc

Question 1

Craniopharyngioma characteristics (4)

Answer 1

1) Growth failure
2) HA
3) Visual field deficit
4) Endocrine abnormalities (dec GH / ADH / thyroid)

Question 2

Primitive neuroectodermal tumors characteristics (4)

Answer 2

1) Most common malignant brain tumor in children
2) Often in cerebellum (called medulloblastoma)
3) Ataxia
4) Inc ICP 2/2 obstructive hydrocephalus

Question 3

Subependymal giant cell astrocytoma characteristics (4)

Answer 3

1) A/w tuberous sclerosis
2) Located in walls of lateral ventricles
3) can cause obstructive hydrocephalus
4) Respond to mTOR inhibitors

Question 4

Mentzer index formula? Cutoff for neta thal trait?

Answer 4

1) MCV / RBC count

2) <11

Question 5

Incidence of fever in pRBC tranfusion? Platelet transfusion?

Answer 5

1) 1%

2) 10%

Question 6

Combination of inc RDW + inc MCHC is nearly 100% specific for?

Answer 6

Hereditary spherocytosis

Question 7

Gold standard for confirming hereditary spherocytosis? And caveat?

Answer 7

Osmotic fragility test 2) Normal in 20-30% of pts w/dx

Question 8

Most common site of metastasis in Ewing’s, rhabdomyosarcoma and germ cell tumors?

Answer 8

Lungs

Question 9

Peak incidence of Burkitt’s lymphoma is in what age group?

Answer 9

5-10 yrs of age

Question 10

Transient erythroblastopenia of childhood:

1) Age group
2) Preceding sx?
3) Tx?
4) Ddx (4)?

Answer 10

1) Ages 1-3
2) Viral illness in the prior 6-8 weeks
3) Monitoring
4) Malignancy, IDA, aplastic anemia, Diamond-Blackfan