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Table 2 Hematological, immunological features and summary of medical management

From: Description and outcome of a cohort of 8 patients with WHIM syndrome from the French Severe Chronic Neutropenia Registry

UPN ANC ALC AMC PLTs Ig G Ig A Ig M CD3 CD4 CD8 CD19 GCSF
  ×109/L ×109/L ×109/L ×109/L g/L g/L g/L ×109/L ×109/L ×109/L ×109/L ATB
             Ig
             Other
5546 0.16 0.38 0.08 223     0.33 0.2 0.1   No GCSF
             No ATB
             No Ig
5449 0.16 2.08 0.16 452 11.6 (6 mo) 0.12 0.199 1.33 0.89 0.35 0.16 No GCSF
             No ATB
             No Ig
Foetus NR NR NR NR NR NR NR NR NR NR NR NR
5780 0.16 1.27 0.1 367 2.83* 0 0.53 1 0.77 0.13 0.07 GCSF short course / efficient
             No ATB
             Early Ig+
5592 1.32 0.3 .021 60 - 0.8 0 0.1 0.02 0.02 0.09 GCSF since age 5 years, on demand since age 8 years / repeated infections despite GCSF :
             ATB+
             Ig +
             Warts treated by surgery and imiquinod with no efficiency
7012 0.22 0.58 0.09 202 9.69 1.89 <0.25 0.67 0.30 0.42 0.01 No GCSF
             No ATB
             No Ig
5231 0.4 0.17 0.07 212 4.2 0.5 0.4 0.3 0.04 0.04 0 GCSF since age 28 years /repeated infections despite GCSF
             No ATB but Aciclovir prophylaxis
             Ig +
5446 0.34 0.42 0.12 193 7.2 0 0 0.33 0.15 0.19 0.04 GCSF since age 34 years / no effect on bronchitis Withdrawn
             ATB+
             Ig +
5447 0.14 1.2 0.1 306 1.8** 1.0 0 0.6 0.54 0.04 0 No GCSF
             No ATB
             Ig +
  1. ALC: Absolute lymphocyte count; AMC: Absolute Monocyte count; ANC: Absolute neutrophil count; GCSF: Granulocyte colony stimulating factor; Ig: Immunoglobulin prophylaxis; NR: Non reported; PLTs: Platelets; UPN : Unique patient number; + Present; - Absent **Antibody against vaccine: normal titers.