Failure

Question 1

Congenital failure syndromes

Answer 1

Fanconi Anemia (FA)
Dyskeratosis Congenita (DC)
Congenital Amegakaryocytic Thrombocytopenia (CAMT) in aplastic phase. Schwachman–Diamond Syndrome.
Blackfan Diamond Anemia (Congenital hypoplastic anemia).

Question 2

DDx for aplastic anemia

Answer 2

Hypocellular Refractory Cytopenia of unknown significance Hypoplastic MDS or acute leukemia
Pure Erythroaplasia
Pure white cell aplasia
T-LGL-leukemia with pancytopenia
Congenital marrow failure syndromes

Question 3

How to Grade AA

Answer 3

ANC
0-0.2 v severe
0.21-0.5 sévère
> 0.5 non severe

Question 4

What infection do 5-10% saa have

Answer 4

Séronégatif hepatitis

Question 5

Best lineage to differentiate hypolplastic mds from severe aa

Answer 5

Megakaryocytes

Question 6

Work up pra

Answer 6

Ct thymoma
Viral incl parvovirus
Ana
TCR réarrangé - look for lGL most common cause PRA

Question 7

PRA definition

Answer 7

normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow