Table 1.

Characteristics and course of common variable immunodeficiency (CVID) in the studied patients

Patient number	1	2	3	4	5	6	7
Age of diagnosis: CVID	6 years	8 years	3 years 6 months	12 years	10 years	8 years	5 years
Main symptoms of CVID	infections of respiratory tract, lymphadenopathy splenomegaly	thrombocytopaenia splenomegaly	severe thrombocytopenia lymphadenopathy splenomegaly	thrombocytopaenia thrombocytopaenia leukopaenia	thrombocytopaenia	pneumonias lymphadenopathy, splenomegaly
Laboratory data at diagnosis of CVID	low level of IgG IgA, low number of T cells, reverse CD4:CD8 ratio, weak response to vaccines	low level of IgG, IgA, IgM, low number ofT,B,andNK cells	low number of T cells, lack of response T cells to stimulation, low level of IgG	low level of IgG, IgA and IgM, low number of T, B and NK cells	low level of IgG	low level of IgG, IgA	low level of IgG, IgM, lack of IgA, leukopaenia, weak response to vaccines
Other symptoms	mild leukopaenia	leukopaenia	severe haemolytic anaemia	severe leukopaenia	no	no	agenesis of kidney, LIP, lung fibrosis (progression), bronchiectases
Response to IgG	good, no infections	good	good	weak (increase of platelets 2-3 days)	good	good	weak (low level of IgG)
Complications	adverse reaction to IVIG, shift to SCIG						severe osteoporosis, overweight, delay of puberty
Other therapies	steroids before IVIG						steroids, azathioprine prolonged because of lung fibrosis progression
Present status	SCIG	IVIG, LIP diagnosis	no substitution, hematologic care	IVIG, hematologic care	no substitution no symptoms	no substitution no symptoms	SCIG, lungs and kidney insufficiency, splenomegaly

LIP - lymphocyte infiltrating pneumonia, IVIG - intravenous substitution of IgG, SCIG - subcutaneous form of substitution