Table 1.

Differential diagnosis between DBA and transient TEC

CharacteristicDBATEC
Median age at diagnosis 2 months >1 year 
Inheritance Sporadic (55%) or dominant (45%) Not inherited 
Congenital anomalies In 50% None 
Pure red blood cell aplasia (bone marrow biopsy) or erythroblastopenia (bone marrow aspiration) Yes Yes 
Hb level Low Low 
Reticulocyte count <20 × 109/L <20 × 109/L 
MCV Usually high Normal 
eADA activity Normal to high Normal 
HbF Normal to high Normal 
Allelic variation in a RP gene or another gene involved in DBA-like cases 70% to 80% of the patients with DBA No mutation found 
CharacteristicDBATEC
Median age at diagnosis 2 months >1 year 
Inheritance Sporadic (55%) or dominant (45%) Not inherited 
Congenital anomalies In 50% None 
Pure red blood cell aplasia (bone marrow biopsy) or erythroblastopenia (bone marrow aspiration) Yes Yes 
Hb level Low Low 
Reticulocyte count <20 × 109/L <20 × 109/L 
MCV Usually high Normal 
eADA activity Normal to high Normal 
HbF Normal to high Normal 
Allelic variation in a RP gene or another gene involved in DBA-like cases 70% to 80% of the patients with DBA No mutation found 

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