DYSGAMMAGLOBULINEMIA: REPORT OF A CASE WITH A FAMILY HISTORY OF A CONGENITAL GAMMA GLOBULIN DISORDER

Abstract

An additional male child with dysgammaglobulinemia is reported. In this instance an older sibling, deceased, had clinical and histopathologic findings suggestive of a congenital gamma globulin deficiency. The preponderance of males among recognized examples of dysgammaglobulinemia, and the presently reported patient whose male sibling also appears to have had a congenital gamma globulin deficiency, suggest that dysgammaglobulinemia, in this particular family, was transmitted as a sexlinked recessive characteristic. It remains unclear whether agammaglobulinemia and dysgammaglobulinemia represent two closely related genetic abnormalities, or whether they represent variable expression of a single genetic disorder. Study of additional pedigrees containing examples of dysgammaglobulinemia will be required to answer this question.