Immune Deficiency Syndromes | B-Cell

sqadia.com gives you the high-yield points you need to know for the various Immune Deficiency Syndromes.

This immunology video covers the immune system and the different types of disorders that result from the loss or dysfunction of the immune cells. Immune responses are of two types, innate and acquired or adaptive immunity. If the components of either of these responses are affected, immune deficiency syndrome occurs. We will look at both responses in detail.

In this video, we will talk about:
🔹B-Cell Immune Deficiency Syndromes
🔹T-Cell Immune Deficiency Syndromes
🔹Innate Immune Deficiency Syndrome

▬ 📌 Immune Deficiency Syndromes
The immune system is a complex network of organs, cells, and proteins that defend the body against infection, whilst protecting the body's own cells. An immune deficiency – which is your immune system not working at its optimum level is the loss or dysfunction of an important component of the immune system.

The immune system is interconnected, and different mechanisms come together to fight off pathogens and develop immunity. Inflammation is a physiological process that helps in fighting off foreign invaders. It can occur because of tissue injury or infection. We have discussed the different mechanisms that bring about immune response. Any dysfunction of the components of these immune responses would lead to the development of deficiency syndrome

▬ 📌 B-Cell Immune Deficiency Syndromes
In this video, we will be going through several Syndromes that develop due to dysfunction in the maturation of B cells.

👉 X-Linked Agammaglobulinemia
Which is due to the failure of B cell precursors to become B cells.

👉 Selective IgA Deficiency
This is due to the deficiency of IgA which is vital in protecting against pathogens.

👉 Common variable immunodeficiency
A collection of many disorders with variable degrees of immunodeficiencies.

▬ 📌 T-Cell Immune Deficiency Syndromes
The T cell, which is a cell of adaptive immunity, matures in the thymus. Several immunodeficiency disorders occur due to loss of the thymus.

Types of adaptive immunity: Humoral-mediated and Cell-Mediated Immunity

👉 Thymic Aplasia
Also known as DiGeorge Syndrome. Where the thymus fails to form, and a classic triad is seen in children which we discussed in the video.

👉 Hyper-IgE Syndrome
This presents with immune symptoms along with skin and bone findings.

👉 Chronic mucocutaneous candidiasis
Due to a defect in the AIRE genes. People with this condition get recurrent candida infections

👉 Severe Combined Immunodeficiency
This is the loss of cell-mediated and humoral immunity so basically the entire adaptive immune symptoms

👉 Ataxia Telangiectasia
This is an autosomal recessive genetic condition where there is a defective ATM gene on chromosome 11

👉 Hyper-IgM Syndrome
This is a class-switching disorder. Failure of B cells to bind to T cells.

👉 Wiskott – Aldrich Syndrome
This is a very rare X-linked disorder of the WAS gene which codes for the WAS protein.

▬ 📌 Innate Immune Deficiency Syndromes
The innate immunodeficiency disorders are developed due to loss of innate immunity. The three immunodeficiency syndromes discussed in this video are:

👉 Leukocyte Adhesion Deficiency
People with this disorder have defective neutrophil and lymphocyte migration.

👉 Chediak – Higashi Syndrome
This is the failure of lysosomes to fuse with phagosomes.

👉 Chronic Granulomatous Disease
In this disease, there is a loss of function of NADPH oxidase.

You can check out the full video where we go through the various Immune Deficiency Syndromes that are important for clinics as well as boards.

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