Inherited and Acquired Bone Marrow Failure Need-to-Know Flashcards Preview

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Flashcards in Inherited and Acquired Bone Marrow Failure Need-to-Know Deck (10):
1

What is the problem in dyskeratosis congenita?

telomere defects

2

Signs of dyskeratosis congenita?

bad fingernails
premature grey hair

3

What is the problem in Fanconi anemia?

DNA repair

4

Signs of Fanconi anemia?

1/3 normal
2/3 bone changes, extra thumb, short, small head, cafe au lait spots

5

What is the test for Fanconi anemia?

DEB test
ox stresses cells

6

Why do you do a DEB test?

you can't give chemo until you know your patient doesn't have a DNA defect (ie/Fanconi anemia)

7

What is the cause of acquired aplastic anemia?

immune mediated response, T cells attack progenitor cells

8

What is the preferred treatment for severe aplastic anemia?

HLA matched sibling BM transplant
under age 25/30
DO DEB TEST FIRST

9

Second/third line Tx for aplastic anemia?

2)intensive immunosuppreive theory (ATG+CSA+Prednisone)
3)unrelated donor BMT

10

Camitta criteria

Peripheral blood (2o3)
-PMN <20,000
BM: marked hypocellularity