This includes, but is not limited to, congenital agammaglobulinemia, common variable immunodeficiency, X-linked agammaglobulinemia, Wiskott-Aldrich syndrome, and severe
combined immunodeficiencies.?
Her newborn son Exander was diagnosed with a rare and potentially life threatening disorder called SCID (Severe
Combined Immunodeficiencies).
Leaky SCIDs and other forms of
combined immunodeficiencies with an indolent but severe prognosis represent a special challenge to geneticists, immunologists, and clinicians involved in the care of these patients.
OPV immunization has been associated with poliovirus infection in patients with primary antibody deficiencies and
combined immunodeficiencies, which can lead to paralysis (7,8).
Octagam is indicated for treatment of primary humoral immunodeficiency (such as congenital agammaglobulinemia), common variable immunodeficiency, X-linked agammaglobulinemia, Wiskott-Aldrich syndrome, and severe
combined immunodeficiencies.
Other
combined immunodeficiencies are part of well-defined immunodeficiency syndromes (e.g., Wiskott-Aldrich syndrome [WAS], ataxia telangiectasia, and hyper-IgE syndrome), all of which are associated with recurrent infections and decreased life expectancy (Table 1).
CMC is frequently a component of
combined immunodeficiencies (with decreased T-cell number or function) in which susceptibility to various infectious agents and noninfectious signs such as autoimmunity is expected.
Of 946 patients with primary immunodeficiency, including 29 with severe
combined immunodeficiencies, diagnosed from 1980 through 2006 at the Children's Memorial Health Institute in Warsaw, adverse events after BCG vaccination were observed in 16 (8,9).