Intravenous immunoglubulin (IVIG) has been the mainstay of treatment for a number of primary immunodeficiencies since it was first approved by the Food and Drug Administration (FDA) in the early 1980s.
In the case of children with XHIM, IVIG is given to replace the missing IgG antibodies and to reduce or normalize the IgM level.
In one Japanese study, five out of seven patients who received BMT survived, with four of the five producing T cells with normal CD40 ligand without supplementary IVIG therapy.
In general, children who are treated with IVIG and/or BMT as infants have a better prognosis than those who are diagnosed after the age of two years.
Children who are receiving IVIG treatment can attend a regular school and participate in most sports provided that minor injuries are treated promptly.