Table 1

Presenting symptoms and features of XLP1 patients with associated mortality

IncidenceMortality
Presenting symptom   
    HLH 31.9% 65.5% 
    FIM 7.7% 14.3% 
    Lymphoma 14.3% 7.7% 
    Dysgammaglobulinemia 22% 5% 
    Family history of XLP1 alone 16.5% 20% 
    Other 7.7% 14.3% 
Features occurring at any time   
    HLH 35.2% 65.6% 
    FIM 9.9% 22.2% 
    Lymphoma 24.2% 9% 
    Dysgammaglobulinemia 50.5% 13% 
    Other 15.4% 28.6% 
IncidenceMortality
Presenting symptom   
    HLH 31.9% 65.5% 
    FIM 7.7% 14.3% 
    Lymphoma 14.3% 7.7% 
    Dysgammaglobulinemia 22% 5% 
    Family history of XLP1 alone 16.5% 20% 
    Other 7.7% 14.3% 
Features occurring at any time   
    HLH 35.2% 65.6% 
    FIM 9.9% 22.2% 
    Lymphoma 24.2% 9% 
    Dysgammaglobulinemia 50.5% 13% 
    Other 15.4% 28.6% 
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