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Flashcards in Aplastic Anemia Deck (13):
1

What causes aplastic anemia?

 

Too few hematopoieic progenitor cells

 

2

What does Acquired Aplastic Anemia need to be differentiated from?

 

Inherited bone marrow failure syndrome

 

3

What causes the destruction of progenitors in bone marrow in SAA?

 

Assault by the T-cells

4

What symptoms are associated with AA?

 

·         Lethargy

·         Epistaxis – Extended time

·         Bacterial infections

5

What triggers are for acquired SAA?

·         Usually not identified

·         Check for CMV, EBV, HHV-6, Parvovirus, Hepatitis

·         History of jaundice

·         Medication history – Chloramphenicol

·         Exposures (Radiation/Nuclear)

6

What is the Camitta Criteria?

 

Severe AA (SAA) “marked pancytopenia”

Peripheral Blood (2 of 3):

  •  < 500 PMN
  • < 20,000 Platelets
  • < 20,000 

BM: 65% hypocellularity

 

7

How is AA treated?

 

Matched Sibling BM 

Intensive Immune Suppression with ATG/CSA/Prednisone

  • Alemtuzumab
  • Cyclophosphamide

Unrelated donor 

Alternative, Alternative Donor BMT

  • Halpo/Cord BMT; Double Cord BMT

Eltrombopag – TPO agonist, but also activates all erythroblasts

8

What are telomeres?
What is the significance of their length?
What do mutations of telomerase cause?
What happens without telomerase?
 

Repeat sequences at ends of chromosomes

Molecular mechanisms have evolved to maintain telomere 

Mutations in telomerase can result in marrow failure, liver fibrosis, lung firbrosis

Without telomerase, somolescence

9

What is the classic disease of cancer biology?

 

Dyskeratosis Congenita

 

10

What symptoms/findings are associated with Dyskeratosis Congenita?
What diagnostic parameters can be used to diagnose DC?
What is the treatment?

 

Leukoplakia, Lacy pigmentation, Abnormal finger and toe 

Telomere length
Telomere length analysis is becoming a standard test
Genetic testing commercially available

Will need a transplant

 

11

What is Fanconi Anemia caused by?
What special test must be done for it and why?
How is it treated?

DNA repair defect in Fanconi Gene 

40% without physical stigmata so must do diagnostic/functional DEB test to look for chromosome 
If DEB test abnormal, genetic testing to classify 

Will need a transplant

12

What is Schwachman-Diamond Syndrome?
What is it caused by?
What clinical findings are there?
What screening tests are there?

 

BM defect due to SBDS gene defect
Exocrine pancreatic deficiency and neutropenia
Isoamylase and trypsinogen are easily screening tests

 

13

What is Diamond-Blackfan Anemia?
What is severe congenital neutropenia?
 

No RBCs
Lack of PMNs congenitally