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Type I hypersensitivity
Autoimmune hemolytic anemia
Hemolytic disease of the newborn
Rheumatic heart disease
Type II hypersensitivity
Systemic lupus erythematosus
Type III hypersensitivity
Type IV hypersensitivity
Common variable immunodeficiency
Hyperimmunoglobulin E syndrome
IgG subclass deficiency
Isolated primary immunoglobulin M deficiency
Selective immunoglobulin A deficiency
Adenosine deaminase deficiency
Hyper IgM syndrome
Severe combined immunodeficiency
Cytomegalovirus infection after transplant (NORD)
Post-transplant lymphoproliferative disorders (NORD)
Chronic granulomatous disease
Leukocyte adhesion deficiency
Blood transfusion reactions and transplant rejection: Pathology review
Immunodeficiencies: Combined T-cell and B-cell disorders: Pathology review
Immunodeficiencies: Phagocyte and complement dysfunction: Pathology review
Immunodeficiencies: T-cell and B-cell disorders: Pathology review
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Hyper IgM Syndrome
Combined B & T-Cell Disorders
hyper-IgM syndrome and p. 115
hyper-IgM syndrome p. 115
Hyper IgM syndrome is a problem where B cells are unable to undergo antibody class-switching, meaning that they can produce IgM antibodies, or immunoglobulins, but struggle to produce other types of antibodies, and that leaves individuals at risk for certain infections.
Let’s take a look at how B cells end up secreting different types of antibodies. Each B cell is born in the bone marrow from a stem cell and develops its own B cell receptor, which sits on the cell surface. The B cell receptor consists of two parts - a protein called CD79 that communicates with the rest of the cell and a membrane bound IgM or IgD antibody that can bind to an antigen. An antigen is any substance recognized by that particular antibody.
Each antibody has two identical light chains and two identical heavy chains that combine into a Y shape. So this Y-shaped antibody’s got two arms with identical tips, which is called the variable region. This variable region contains an antigen binding domain that’s unique to that antibody.
Below the variable region, or toward the point where the arms meet, is the constant region where every member of an antibody class is identical – so all IgM antibodies have the same constant region, but IgM and IgA constant regions are different.
And there are five classes of antibodies in total: IgM, IgG, IgA, IgE, and IgD class antibodies. And each antibody class has a slightly different job. For example, IgMs are part of B cell receptors, and are the first free-floating antibodies produced in an immune response. They’re secreted as a pentamer, meaning there are five antibodies connected together, which provides many binding sites for grabbing antigens and taking them out of the blood. Each antibody has complement protein binding sites on the heavy chains, so these IgM pentamers are also great at activating complement proteins, which help destroy and remove pathogens.
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