What is Hypogammaglobulinemia?

I’ve recently been diagnosed with the immune disorder Hypogammaglobulinemia which requires the expertise of an Endocrinologist. My doctor isn’t sure how I contracted it since I don’t fit any noted categories. 

Infusion treatments may become necessary for Hypogammaglobinemia, some patients only require one treatment and others require ongoing infusion treatment for life.

Overview

Hypogammaglobulinemia is a problem with the immune system that prevents it from making enough antibodies called immunoglobulins. Antibodies are proteins that help your body recognize and fight off foreign invaders like bacteria, viruses, and fungi.

Without enough antibodies, you’re more likely to get infections. People with Hypogammaglobulinemia can more easily catch pneumonia, meningitis, and other infections that a healthy immune system would normally protect against. These infections can damage organs and lead to potentially serious complications.

Causes

Several gene changes (mutations) have been linked to Hypogammaglobulinemia.

One such mutation affects the BTK gene. This gene is needed to help B cells grow and mature. B cells are a type of immune cell that makes antibodies. Immature B cells don’t make enough antibodies to protect the body from infection.

THI is more common in premature infants. Babies normally get antibodies from others through the placenta during pregnancy. These antibodies protect them from infections once they’re born. Babies that are born too early don’t get enough antibodies from their mothers.

A few other conditions can cause Hypogammaglobulinemia. Some are passed down through families and start at birth (congenital). These are called primary immune deficiencies.

They include:

• ataxia-telangiectasia (A-T)
• autosomal recessive agammaglobulinemia (ARA)
• common variable immunodeficiency (CVID)
• hyper-IgM syndromes
• IgG subclass deficiency
• isolated non-IgG immunoglobulin deficiencies
• severe combined immunodeficiency (SCID)
• specific antibody deficiency (SAD)
• Wiskott-Aldrich syndrome
• x-linked agammaglobulinemia

More oftenTrusted Source, Hypogammaglobulinemia develops as a result of another condition, called secondary or acquired immune deficiencies. These include:

• blood cancers such as chronic lymphocytic leukemia (CLL), lymphoma, or myeloma
• HIV
• nephrotic syndrome
• poor nutrition
• protein-losing enteropathy
• organ transplant
• radiation

Certain medications can also cause hypogammaglobulinemia, including:

• medicines that suppress the immune system, such as corticosteroids
• chemotherapy drugs
• antiseizure medications

Treatment options

If your Hypogammaglobulinemia is severe, you may get Immune Globulin replacement therapy to replace what your body isn’t making. You get this treatment through an IV. The immune globulin comes from the blood plasma of healthy donors.

I’ll keep you posted. 

Melinda