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

Heme/Lymph > Inherited and Acquired Bone Marrow Failure Need-to-Know > Flashcards

Flashcards in Inherited and Acquired Bone Marrow Failure Need-to-Know Deck (10)
Loading flashcards...
1
Q

What is the problem in dyskeratosis congenita?

A

telomere defects

2
Q

Signs of dyskeratosis congenita?

A

bad fingernails

premature grey hair

3
Q

What is the problem in Fanconi anemia?

A

DNA repair

4
Q

Signs of Fanconi anemia?

A

1/3 normal

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

5
Q

What is the test for Fanconi anemia?

A

DEB test

ox stresses cells

6
Q

Why do you do a DEB test?

A

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

7
Q

What is the cause of acquired aplastic anemia?

A

immune mediated response, T cells attack progenitor cells

8
Q

What is the preferred treatment for severe aplastic anemia?

A

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

9
Q

Second/third line Tx for aplastic anemia?

A

2) intensive immunosuppreive theory (ATG+CSA+Prednisone)

3) unrelated donor BMT

10
Q

Camitta criteria

A

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