Abstract
A four year old girl (consanginous parents) with primary macroglobulinemia was followed for 3 years. She presented with cogenital hypotonia, recurrent infections, splenornegally, eosinophilia and increasing serum IgM to 4 grams percent. The serum protein electrophorectic pattern revealed three distinct gamma spikes, two of which were seen in the concentrated urine. A high molecular weight IgM with both kappa and lambda determinants was isolated from the serum indicating at least a biclonal dyscrasia. More lambda than kappa light chains were associated with the IgM on immunoelectrophoresis. The abnormal IgM has euglobulin, cryoglobulin, absent isohemagglutinins, and high anti-IgG activities with low serum complement levels. The initially normal serum IgA fell to zero. Impaired IgG antibody responses were found. Frogressive cell-mediated immune deficiency is suggested by an uncomplicated smallpox vaccination at one year followed by persistent mucocutanous candidiasis with a negative delayed skin test to Candida and a impaired PHA response of the peripheral lymphocytes at 4 yrs.. This is the first described case of primary macroglobulinemia in a child. The abnormal serum IgM level progressively fell with chlorambucil therapy.
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Reid, R., Weiss, L., Fraher, T. et al. PRIMARY MACROGLOBULINEMIA IN A CHILD. Pediatr Res 8, 418 (1974). https://doi.org/10.1203/00006450-197404000-00466
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DOI: https://doi.org/10.1203/00006450-197404000-00466