Table 4

Vasculitis in patients with severe WAS in the first 2 years of life

PatientManifestation(s)Age, moTherapy
P5 Joints, skin, gut 22 IV CS → CR → relapse → CR → HSCT 
P7 Brain 11 IV CS → PR → CS + Cy → CR → HSCT 
P9 Joints, skin 14 IV CS → CR → AIHA → HSCT 
P15 Lungs, skin IV CS → CR → HSCT 
P17 Joints, skin, high fever 17 IV CS → CR → AIHA → HSCT 
P18 Joints, skin, high fever IV CS → PR → CS + IVIgs → CR → HSCT 
PatientManifestation(s)Age, moTherapy
P5 Joints, skin, gut 22 IV CS → CR → relapse → CR → HSCT 
P7 Brain 11 IV CS → PR → CS + Cy → CR → HSCT 
P9 Joints, skin 14 IV CS → CR → AIHA → HSCT 
P15 Lungs, skin IV CS → CR → HSCT 
P17 Joints, skin, high fever 17 IV CS → CR → AIHA → HSCT 
P18 Joints, skin, high fever IV CS → PR → CS + IVIgs → CR → HSCT 

Cy indicates cyclophosphamide.

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