Summary Six cases with Aldrich syndrome are presented, one of them splenectomized at the age of 3, died at the age of 6 years of adrenal haemorrhage. Another patient died at the age of 6 years of pneumonia with empyema and meningitis. The other 4 patients (1/2–6 years) are alive in a fairly good condition. Bleeding tendency dominates the symptoms in 2 cases, in the other 2, susceptibility to infections and/or eczema are the main symptoms. The bleeding tendency does not seem to be correlated to the number of platelets. Immunological studies in 5 of the patients have revealed a normal or high content of IgG in 4 of 5 cases and an increase of IgA in all. In 3 of 4 investigated patients elevated concentrations were demonstrated of a new immunoglobulin class, IgND. The immune response is changed; antibody production by vaccinations against viral diseases was poor, tuberculin reactions after BCG vaccination were negative, the pharyngeal lymphoid tissue was absent, and in 4 of the 5 investigated cases low titers of isoagglutinins were demonstrated.
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Berglund et al. (1968) studied this question.
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