Table 1.

Clinical data on patients whose DCs showed poor expression of MHC Class II-DR on initial testing. Family members of 15 of the 25 patients in the cohort were screened for immunoglobulin deficiencies. Five patients had affected family members with CVID and/or selective IgA deficiency (IgAD), 4 of whom appear in this table.

		Serum immunoglobulins
Patient no.	Age (years)	IgG	IgA	IgM	Familial CVID/IgAD	Complications	No. of repeat tests
3	50	<2·0	<0·1	<0·1	Yes	Granulomatous	1 (ad)*
						lung disease	2 (col)†
8	57	4·5	<0·1	0·6	ND	Bronchitis	1 (ad)
							3 (col)
13	48	4·1	0·4	0·6	Yes	Severe bronchiectasis	1 (ad)
							2 (col) (N)
17	51	2·2	<0·2	0·4	ND	Severe enteropathy	1 (ad)
							2 (col)
20	38	4·4	<0·1	0·3	Yes	Very high	1 (ad)
						anti-IgA antibodies	3 (col)
25	44	<1·5	<0·1	<0·1	Yes	Gastritis with	2 (ad)
						vitamin B12 deficiency	2 (col)

^*ad, MdDC-adh;

^†CD14 MdDC-Col ND, Family members not screened N denotes the only patient (no. 13) whose DCs showed normal DR expression when subsequently tested using column separated monocyte precursors.