Primary Acquired Agammaglobulinemia, with a Family History of Collagen Disease and Hematologic Disorders

Abstract

PRIMARY agammaglobulinemia was once thought to be an extremely rare condition. However, since Bruton¹ first recognized the syndrome in 1952, nearly 200 cases have appeared in the literature. These fall into three pathological categories: primary congenital agammaglobulinemia, which is a sex-linked recessive trait and usually becomes manifest late in the first year of life^{2 3 4}; primary acquired agammaglobulinemia, which has no clear-cut genetic basis and may appear at any time in life; and secondary agammaglobulinemia, which is a symptom, found in certain neoplastic diseases, such as Hodgkin's disease, and other malignant lymphomas, multiple myeloma and chronic lymphatic leukemia.^{5 6 7} The syndrome . . .