Fatal progressive vaccinia in two immunodeficient infants.

Fatal progressive vaccinia developed in two infants, a girl and a boy, vaccinated at the age of 2 months. Immunodeficiencies comprised both humoral and cell-mediated immunity. In the girl low levels of immunoglobulins and a defect function of lymphocytes was demonstrated. The boy had hypogammaglobulinemia and lack of T-lymphocytes. There was a marked hypoplasia of thymus and lymphoid organs in both infants. Both infants had the haplotype A3, B8 and in the boy a crossing over had taken place within the HLA region. No effect was achieved with antivaccinia immunoglobulin and concomitant antiviral therapy (thiosemicarbazone, adenine arabinoside). Interferon therapy gave no clinical improvement, nor did transfer factor.