Immunologically Distinct Forms of Primary Hypergammaglobulinaemia: Studies Using Pokeweed Mitogen and Epstein-Barr Virus
Since the first demonstration that some patients with repeated attacks of respiratory tract infection lacked serum immunoglobulins, there have been many attempts to understand the immunological basis for this deficiency. Some of the patients present with infections within the first year of life and many of these show an X-linked pattern of inheritance, X-linked agammaglobulinaemia or XLA. The boys and young men with XLA are in many ways distinct from the patients who present after the age of 2 years who show no sex bias and usually no family history; common variable immune deficiency or CVID. There are also a few cases where hypogammaglobulinaemia is associated with the presence of a thymoma, these cases generally present over the age of 45 years and they have a much worse prognosis. The investigation of all these patients has changed progressively over the last 10 years in parallel with advances in our general understanding of antibody production.
KeywordsCord Blood Immune Deficiency Cellular Immunity Suppressor Cell Common Variable Immune Deficiency
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