Immunodeficiency disorders - Primary: Nursing

Primary immunodeficiency disorders are a diverse group of conditions that affect one or more elements of the immune system, leading to increased vulnerability to infection, autoimmune manifestations, and several types of cancer. All right, let’s quickly review some physiology. The immune system consists of white blood cells that protect us from pathogens like viruses, bacteria, and fungi, as well a foreign substances, such as toxins and chemicals, and destroy abnormal cells, such as those that might develop into cancer. Now, the immune system consists of two main branches: innate and adaptive. The innate immune response involves non-specific defense mechanisms, meaning that they do not differentiate one pathogen from another. These include complement proteins and cells like phagocytes and natural killers; as well as dendritic cells, which then activate the adaptive immune response.

The adaptive response is highly specific, meaning that it recognizes different pathogens and is mediated by cells called lymphocytes, which include T and B cells. T cells can be further divided into CD4+ and CD8+ T cells. CD4+ T cells are also known as T helper cells, because they interact with dendritic cells, and in turn help activate the rest of the lymphocytes. On the other hand, CD8+ T cells, also known as cytotoxic T cells, are in charge of cell-mediated immunity, where they attack abnormal and infected cells. Finally, B cells mediate a specific adaptive response, called humoral immunity, by secreting antibodies that bind to and destroy extracellular pathogens. These antibodies can be classified into several classes based on their structure and function, including IgM, IgA, IgD, IgG and IgE. Now, primary immunodeficiencies are a group of over 130 disorders that result from genetic defects in one or more elements of the immune system, including antibodies, T cells, complement components, and phagocytes.