Pedia: Hema Onco

Question 0

Pure red cell aphasia is also known as?

Answer 0

Diamond Blackfan Syndrome

Question 1

Name 4 viruses that may cause aplastic anemia.

Answer 1

Parvovirus B19, Hepatitis, HIV, EBV

Question 2

How to dfx IDA from anemia of chronic dz based on TIBC?

Answer 2

IDA has high TIBC.

Question 3

Hemoglobin H dz has how many foci deleted or mutated to cause alpha thalassemia?

Answer 3

3/4

Question 4

Cooley’s anemia usually manifests at what age?

Answer 4

6 months when hgb F declines

Question 5

What 3 groups of antipathogens cause G6PD?

Answer 5

Sulfa drugs, primaquine, INH

Question 6

The level of metalloproteinase is normal in:
TTP
HUS

Answer 6

HUS

Question 7

Vonwillebrand dz and hemophilia A can both be treated by which artificial hormone?

Answer 7

Swamp pressing

Question 8

What is the most common inherited bleeding disorder?

Answer 8

Von wille brand dz also associated with factor 8 deficiency because VW carries f8

Question 9

What leukemia is most common in:

1. Pediatric population
2. 15- 39yo
3. 40-60
4. More than 60

Answer 9

1. ALL
2. AML
3. AML or CML
4. CLL

Question 10

Vit A can be treat which type of leukemia?

Answer 10

Acute promyelocytic leukemia, a subtype of AML

Question 11

Stage 4S if neuroblastoma is usually treated with

Answer 11

Observation

Question 12

Nephroblastoma/ neuroblastoma?

1. Ass with WAGR
2. Ass with Denys Dash
3. Imaging reveals calcified
4. hemorrhagic components
5. Small round blue cells
6. Crosses midline

Answer 12

1 and 2 Wilms the rest neuroblastoma

Question 13

What is the most common location of rhabdomysarcoma in the pediatric population?

Answer 13

Head and neck

Question 14

Osteosarcoma or Ewings?

1. Associated with Li Fraumeni
2. Multi laminar periosteal reaction
3. Onion skinning
4. Undifferentiated small round cells
5. Primarily in whites
6. Metaphysis of long bones

Answer 14

1. Osteo
2. Ewing
3. Ewing
4. Ewing
5. Ewing
6. Osteosarcoma

Question 15

Upon giving iron to IDA anemia packets when would you expect them to have subjective improvement?
Increase in retic count?
Increase in Hgb?

Answer 15

Upon giving iron to IDA anemia pxs when would you expect them to have subjective improvement? A day
Increase in retic count? 3 days
Increase in Hgb? 2 months

Question 16

In beta thalassemia what infection is present to eat up excess iron?

Answer 16

Yersinia enterolytica

Question 17

Heinz bodies are to G6PD while Howel Jolly bodies are to?

Answer 17

Sickle cell dz

Question 18

TTP is to ab to ADAMTS 13 resulting in vWF multimers leading to PLT aggregation and thrombosis
As HUS is to?

Answer 18

Toxin against endothelial cells that result in activation

Question 19

What assay measures vWF antigen levels?

Answer 19

Ristocetin

Question 20

Desmopressin may be used for which bleeding problems?

Answer 20

Von will def bud hemo A

Question 21

What percentage of the bone marrow cells need to be lymphoblasts FTP diagnose ALL?
To diagnose remission? To diagnose relapse?

Answer 21

More than 25%
Less than 5
More than 15-20%

Question 22

What meds are used in ALL
Remission induction
Maintenance

Answer 22

R: vincristine prednisone l asaparaginase
M: methotrexate and mercaptopurine

Question 23

Treatment for neuroblastoma in <1 year old, with dissemination to distant sites skin liver WITHOUT none involvement

Answer 23

4s stage observation

Question 24

What is the relationship of radiation to osteosarcoma and Ewing?

Answer 24

Predisposition to osteosarcoma and treatment for Ewing