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. 2019 Aug 13;10:1908. doi: 10.3389/fimmu.2019.01908

Table 6.

Clinical and immunological features of SCID siblings.

Patient ID AO AD DD Gene Onset sign OS ALC cells/μL CD3+ cells/μL CD4+ cells/μL CD8+ cells/μL CD19+ cells/μL CD56+ cells/μL Immunophenotype Outcome Cause of deaths
A1 1 4 3 Unknown Chronic diarrhea - 1100a 972a 720a 306a 18a 616a TlowBNK+ dead Systemic viral infection
A2 0.5 3.5 3 Unknown Erythroderma + 22255 9258 4206 5786 89a 3116 T+BlowNK+ dead OS
B1 2 6 4 FOXN1 Eczema + 5392 2006 1065 439 3950 1442 T+B+NK+ dead Pneumonia
B2 1 1 0 FOXN1 Eczema + 2688 753a 645a 215a 962 650 TlowB+NK+ alive
C1 3 8 5 IL2RG P. Jiroveci pneumonia - 5528 719a 55a 685a 4765 NA TlowB+ dead PH
C2 12 12 0 IL2RG Asymptomatic - 931a 19a 2a 9a 847 7.4a TB+NK alive
D1 3 5 2 JAK3 Chronic diarrhea - 1824a 62a 40a 18a 1621 11a TB+NK alive
D2 2 2 0 JAK3 Asymptomatic - 3926 12a 0a 1a 3299 10a TB+NK alive
a

< normal age range value; ALC, Absolute Lymphocyte count; NA, not available; OS, Omenn syndrome; PH, pulmonary arterial hypertension.