Table 1.

Clinical characteristics for CSR-like CVID patients at presentation

Patient	#1	#2	#3	#4	#5
Year of birth	1989	1939	1958	1965	1944
Gender	Male	Male	Male	Female	Female
Age at diagnosis	18	20	14	41	60
Clinical presentation
	Appendicitis	Rec. lung infections	Bronchiectasis	Bronchiectasis	Rec. lung infections
	Rec. fever	Tonsillitis	Deafness	ITP	Bronchiectasis
	Pneumonia		Mild mental retardation	Splenomegaly
	Sinusitis		Dental caries
Gene analysis
CD40L	neg	neg	neg	neg	neg
CD40	neg	neg	neg	neg	neg
UNG	neg	neg	neg	neg	neg
AICDA	neg	neg	neg	neg	neg
PI3Kdelta	neg	neg	neg	neg	neg
Immunoglobulins at presentation (g/L)
IgG	<0.3	<0.1	1.2	2.5	<0.3
IgM	<0.03	0.46	7.05	5.27	2.5
IgA	<0.04	<0.05	<0.22	<0.22	<0.04
IgE	<2	ND	<2	<2	ND
B cell subsets at presentation
IgD+CD27− (naive)	80.1	55.4	74	92	95.9
IgD+CD27+ (non-switched memory)	12.2	42.8	18.6	5	1.3
IgD−CD27+ (switched-memory)	4.2	1	0	0.7	0.2
IgD−CD27− (double negative)	3.5	0.8	7.4	2.3	2.6

Year of birth, patient gender, age at diagnosis, clinical presentation, mutations (neg tested negative), immunoglobulin levels at presentation, and B cell subset at presentation is shown

ND not done, HUS Hemolytic-uremic syndrome, ITP Idiopathic thrombocytopenic purpura