Flashcards in Inherited and Acquired Bone Marrow Failure Need-to-Know Deck (10)
What is the problem in dyskeratosis congenita?
Signs of dyskeratosis congenita?
premature grey hair
What is the problem in Fanconi anemia?
Signs of Fanconi anemia?
2/3 bone changes, extra thumb, short, small head, cafe au lait spots
What is the test for Fanconi anemia?
ox stresses cells
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)
What is the cause of acquired aplastic anemia?
immune mediated response, T cells attack progenitor cells
What is the preferred treatment for severe aplastic anemia?
HLA matched sibling BM transplant
under age 25/30
DO DEB TEST FIRST
Second/third line Tx for aplastic anemia?
2)intensive immunosuppreive theory (ATG+CSA+Prednisone)
3)unrelated donor BMT