Indication

Primary immune deficiency disorder

A primary immune deficiency disorder encompasses a heterogeneous group of genetically determined conditions, also classified as inborn errors of immunity, that impair the normal development and functioning of the immune system. Read more about Primary immune deficiency disorder

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About Primary immune deficiency disorder.

A primary immune deficiency disorder encompasses a heterogeneous group of genetically determined conditions, also classified as inborn errors of immunity, that impair the normal development and functioning of the immune system.

These disorders affect pediatric and adult populations and stem from intrinsic defects within innate or adaptive immunity, including abnormalities in B cells, T cells, natural killer cells, phagocytes, or the complement cascade.

Pathophysiologically, the resulting immune incompetence leads to hallmark clinical manifestations such as recurrent, prolonged, or opportunistic infections, as well as immune dysregulation characterized by severe autoimmunity, chronic inflammation, lymphoproliferation, and an increased susceptibility to malignancy.

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