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

Table 2 Hematological, immunological features and summary of medical management

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5446	0.34	0.42	0.12	193	7.2	0	0	0.33	0.15	0.19	0.04	GCSF since age 34 years / no effect on bronchitis Withdrawn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 +

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.

ISSN: 1750-1172