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Genetics of immunity deficiency syndromes.

The concept of immunity deficiency implies a definable range of normal immune reactivity from which a significant deviation can be detected and, perhaps, described in detail. In the past 20 years progress in experimental immunology has increased our understanding of both antibody and cellmediated immunity, and of the thymus and lymphoid system as a whole. In clinical medicine a parallel advance, due in part to the prolongation of lives at risk by antibiotics, has led to the recognition of a variety of syndromes of immune deficiency. All are more or less rare but in many a hereditary element is clear and in some the basic pathology of the defect can be analysed. (For classification see Seligmann, Fudenberg, and Good, 1968.) Many uncertainties remain. Thus, whereas most deficiencies are manifest in infancy or childhood and the genetic basis is quite plain, other defects may only be discovered in adult life when hereditary influences may be overlooked. The situation is further confused through the mimicry of genetic disease by infection, e.g. rubella, acquired in utero, and by the obscure role of materno-fetal immunocyte chimerism. Modem analysis of specific immune processes in vertebrates has distinguished a number of components. These include 'effector' cells concerned with both antibody and cell-mediated immunity, as well as 'reactor' (antigen-sensitive) lymphocytes, memory cells, and macrophages. The latter, together with polymorphonuclear neutrophils and those cells that synthesize substances such as leucotaxins, are also primarily responsible for the function of the more primitive processes of nonspecific immunity. The interdependence of all these components and the development of the whole system, comprising cell migration, proliferation, inductive processes, and differentiation, give rise to a large number of steps at which failure may occur but which also afford some opportunity for compensatory balance. The most clearly defined and commonest group of disorders is that of the antibody-forming system,