Impaired antibody response to polysaccharides in association with functional asplenia

Abstract

Impaired antibody responses to polysaccharide antigens despite normal or nearly normal levels of immunoglobulins (including IgG subclasses) have been noted uniformly in patients with Wiskott-Aldrich syndrome and recently in patients without that condition, some of whom also had normal antibody responses to certain protein antigens. 1,2 We present here a boy with similar immunologic findings in whom we found an associated unexplained functional asplenia. CASE REPORT A 21A-year-01d black male child was referred to Duke University Medical Center for immunologic evaluation because of a history of recurrent serious infections. He was the 3180 gm product of a pregnancy complicated only by some maternal weight loss in the third trimester. His neonatal course was notable for the congenital absence or malformation of several fingers on both hands, consistent with the amniotic band syndrome. The patient was first hospitalized at 2 months of age for pneumonia, followed by hospitalizations at 4 months for Haemophilus influenzae meningitis and at 5, 6, and 9 months for episodes of pneumococcal sepsis. Immunoglobulin and isohemagglutinin levels were reportedly normal, and empiric prophylactic amoxicillin therapy was begun. The patient then did fairly well until 29 months of age, when he developed severe disseminated varicella with lung and liver involvement, as well as disseminated intravascular coagulation. He eventually recovered after intravenous acyclovir therapy. METHODS Immunoglobulins G, A, and M were measured by radial immunodiffusion, and specific antibodies to diphtheria and tetanus were measured by tanned cell hemagglutination in our laboratory2 Analysis of lymphocyte proliferation in