Table 1

The first clinical manifestations leading to an assignment of a score of 5 in the first 2 years of life (N = 26)

Manifestation at onset of a score of 5n%
SRT only 12 46 
AIHA only 12 
AIHA + profound thrombocytopenia* 12 
Autoimmune pancytopenia 12 
Vasculitis only 15 
Vasculitis + SRT 
Tumor only 
Manifestation at onset of a score of 5n%
SRT only 12 46 
AIHA only 12 
AIHA + profound thrombocytopenia* 12 
Autoimmune pancytopenia 12 
Vasculitis only 15 
Vasculitis + SRT 
Tumor only 

SRT indicates severe refractory thrombocytopenia (platelet count < 10 G/L); profound thrombocytopenia, a platelet count of between 10 and 20 G/L; and AIHA, autoimmune hemolytic anemia.

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