Common variable immunodeficiency: Clinical and immunological features of 248 patients

Common variable immunodeficiency (CVI) is a primary immunodeficiency diseas e characterized by reduced serum immunoglobulins and heterogeneous clinical features. In these studies we describe the clinical and immunological stat us of 248 consecutively referred CVI patients of age range 3-79 years who h ave been followed for a period of 1-25 years. The median age at the time of onset of symptoms was 23 years for males and 28 years for females; the mea n age at which the diagnosis of CVI was made was 29 years for males and 33 years for females. Forty percent of patients had impaired T cell proliferat ion to one or more mitogens; lymphocyte transformation to mitogens was dire ctly related to the level of the serum IgG. Females at all ages had higher levels of serum IgM than males. Survival 20 years after diagnosis of CVI wa s 64% for males and 67% for females, compared to the expected 92% populatio n survival for males and 94% for females. Parameters associated with mortal ity in this period were lower levels of serum IgG, poorer T cell responses to phytohemagglutinin, and, particularly, a lower percentage of peripheral B cells (P < 0.006). (C) 1999 Academic Press.