Table 1. Clinical and Immunological features of patients with XLA in the present cohort.

Clinical and immunological characteristics	P1	P2	P3	P4	P5
Age at onset	1 year	5 months	7 months	5 months	6 months
Age at diagnosis	7 years	1 year 7 months	1 year 6 months	6 months	8 years
Type of infections	Pneumonia, Pyoderma, Pyogenic meningitis	Pneumonia, Acute suppurative otitis media, Diarrhea, Oral thrush	Pneumonia Diarrhea Pyoderma	Diarrhea	Pneumonia, Pyogenic meningitis Diarrhea, pyoderma
Absent / atrophic tonsils	yes	yes	yes	yes	yes
Sibling death	yes	no	yes	yes	no
No. of hospitalizations before diagnosis	2	>15	1	2	>10
No. of PICU admissions	none	2	1	none	1
Immunoglobulin assay	IgG low, IgA low, IgM low	IgG low, IgA low, IgM normal	IgG low, IgA normal, IgM normal	IgG low, Ig A low, IgM normal	IgG low, IgA low, IgM normal
CD 19 count at diagnosis (N) In cells/mm3	0 (91–610)	0 (430–3300)	8 (430–3300)	10 (430–3300)	10 (91–610)
CD 3 count at diagnosis (N) in cells/mm3	6458 (570–2400)	5475 (1460–5440)	6400 (1460–5440)	4169 (1900–5900)	9553 (570–2400)
CD 56 count at diagnosis (N) in cells/mm3	355 (78–470)	7.0175 (80–340)	160 (80–340)	661 (160–950)	528 (78–470)
Onset to IVIG start	Not on regular IVIG	1 year, 2 months	11 months	1 month	Not on regular IVIG
Complications	Bronchiectasis	None	None	None	Bronchiectasis, Arthritis
Outcome	Stunted growth,	Stunted growth, Cured–HSCT from matched sibling donor (twin) at 6.5 years	Stunted growth Died	Cured-HSCT from matched sibling donor at 2 years	Stunted growth and delayed puberty