121014 aplastic anemia Flashcards Preview

Hem part 2 > 121014 aplastic anemia > Flashcards

Flashcards in 121014 aplastic anemia Deck (14)
Loading flashcards...
1
Q

aplastic anemia is inherited or acquired?

A

can be inherited or acquired

2
Q

PE findings for Fanconi anemia

A

short kids
cafe au lait spots
or asymptomatic

3
Q

aplastic anemia

A

too few hematopoietic progenitor cells leading to too few RBCs, WBCs, platelets

acquired aplastic anemia needs to be differentiated from inherited bone marrow failure syndrome

4
Q

acquired severe aplastic anemia can result from what?

A

viruses
medication
autoimmune

usually don’t find the cause

5
Q

Camitta criteria for severity of aplastic anemia

A

for severe aplastic anemia:

peripheral blood needs to have 2 of the 3:
PMN under 500/uL
platelets under 20,000/uL
reticulocytes under 20,000/uL (corrected retic under 1%)

marrow: marked hypocellularity

6
Q

well recognized inherited marrow failure syndromes?

A

dyskeratosis congenita

Fanconi anemia

7
Q

dyskeratosis congenita

A

telomere disease–accelerated shortening

8
Q

weird, white, thick toenail

A

dyskeratosis congenita

9
Q

Fanconi anemia

A

DNA repair defect

10
Q

what treatments are there for aplastic anemia?

A

matched sibling bone marrow transplant
intensive immune suppression
unrelated donor bone marrow transplant
eltrombopag (thrombopoietin agonist)–to get the “seeds” to work harder

11
Q

what findings can you see for telomere disease?

A

hematology-pulm-hepatic diseases in pedigrees

subtle nail, skin, mucous membrane findings

12
Q

should have what also in your diagnosis for a pt with pancytopenia? ( in addition to differential listed on slide)

A

HLH

13
Q

dyskeratosis symptoms

A

leukoplakia (white patches in oral cavity)
lacy pigmentation
abnormal finger and toe nails
lung and liver disease

14
Q

what test do you do for Fanconi anemia?

A

40% of the pts don’t have physical signs so must do diagnoistic/functional DEB test to look for chromosome breakage

if DEB abnormal, do genetic testing to classify