Congenital immunodeficiency syndromes

Incidence: rare, though as knowledge increases there is recognition of an increasing number of inherited defects in the complex human host defence system. The classical life-threatening disorders of specific immunity with major dysfunction or absence of T cells and/or B cells are all diseases that present in childhood, but milder variants may not be recognized until later life. 'Immunodeficiency' is a vague term that is generally taken to encompass also defects in opsonisation and phagocytosis, so can be taken to include neutrophil and macrophage disorders of number, function or both.

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