Pedia: Hema Onco Flashcards

1
Q

Pure red cell aphasia is also known as?

A

Diamond Blackfan Syndrome

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2
Q

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

A

IDA has high TIBC.

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3
Q

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

A

3/4

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4
Q

Cooley’s anemia usually manifests at what age?

A

6 months when hgb F declines

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5
Q

What 3 groups of antipathogens cause G6PD?

A

Sulfa drugs, primaquine, INH

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6
Q

The level of metalloproteinase is normal in:
TTP
HUS

A

HUS

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7
Q

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

A

Swamp pressing

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8
Q

What is the most common inherited bleeding disorder?

A

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

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9
Q

What leukemia is most common in:

  1. Pediatric population
  2. 15- 39yo
  3. 40-60
  4. More than 60
A
  1. ALL
  2. AML
  3. AML or CML
  4. CLL
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10
Q

Vit A can be treat which type of leukemia?

A

Acute promyelocytic leukemia, a subtype of AML

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11
Q

Stage 4S if neuroblastoma is usually treated with

A

Observation

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12
Q

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
A

1 and 2 Wilms the rest neuroblastoma

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13
Q

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

A

Head and neck

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14
Q

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
A
  1. Osteo
  2. Ewing
  3. Ewing
  4. Ewing
  5. Ewing
  6. Osteosarcoma
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15
Q

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

A

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

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16
Q

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

A

Yersinia enterolytica

17
Q

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

A

Sickle cell dz

18
Q

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

A

Toxin against endothelial cells that result in activation

19
Q

What assay measures vWF antigen levels?

A

Ristocetin

20
Q

Desmopressin may be used for which bleeding problems?

A

Von will def bud hemo A

21
Q

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

A

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

22
Q

What meds are used in ALL
Remission induction
Maintenance

A

R: vincristine prednisone l asaparaginase
M: methotrexate and mercaptopurine

23
Q

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

A

4s stage observation

24
Q

What is the relationship of radiation to osteosarcoma and Ewing?

A

Predisposition to osteosarcoma and treatment for Ewing

25
Q

Name 4 viruses that may cause aplastic anemia.

A

Parvovirus B19, Hepatitis, HIV, EBV