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Hypogammaglobulinemia

 Hypogammaglobulinemia


Overview

Hypogammaglobulinemia is a condition that affects your body’s ability to make immunoglobulins (Ig), or antibodies. Antibodies prompt your body’s immune system to protect you from viruses, bacteria, and other sources of illness. With fewer antibodies available, people with hypogammaglobulinemia have a much higher risk of infection and disease.3

National Cancer Institute. Hypogammaglobulinemia.

Hypogammaglobulinemia is often inherited due to genetic abnormalities. In some cases, people develop hypogammaglobulinemia due to external causes, such as chemotherapy, certain comorbid disorders (those that occur along with hypogammaglobulinemia), or the use of immunosuppressants. Some patients also acquire new mutations (for poorly understood reasons) that lead to the development of this condition.5 Treatment options include antibiotics and/or immunoglobulin replacement therapy to increase antibody levels and improve immune response.

Symptoms

People with this condition get more frequent infections than usual. Common infections include:

* bronchitis

* ear infections

* meningitis

* pneumonia

* sinus infections

* skin infections

* Some of these infections can be serious.

Babies with hypogammaglobulinemia often get respiratory tract infections, food allergies, and eczema. Infants can also develop urinary tract and intestinal infections.

Babies that are born with THI first show symptoms about 6 to 12 months after birth. The main symptom is frequent ear, sinus, and lung infections.

Which symptoms you or your child has will depend on what infections you get, but they can include:

* coughing

* sore throat

* fever

* ear pain

* congestion

* sinus pain

* diarrhea

* nausea and vomiting

* abdominal cramps

* joint pain

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 their mother 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 mother.

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

Diagnosis

Your healthcare provider can make a diagnosis of hypogammaglobulinemia using a physical examination, an assessment of your medical history (such as a history of recurring infections), and a series of lab tests.1 Blood tests may reveal low levels of certain antibodies, such as immunoglobulin G (IgG). You may also develop lower than expected levels of antibodies than expected after vaccination.

 Treatment

If you have secondary hypogammaglobulinemia, treating the underlying cause will help to reduce your symptoms. For example, you may experience fewer symptoms once you stop taking immunosuppressants or after your chemotherapy treatments have ended.

Treatment for primary hypogammaglobulinemia typically involves antibiotics and/or immunoglobulin therapy. 

Antibiotics can help to relieve the symptoms of frequent infections. Your healthcare provider may also prescribe prophylactic antibiotics, which you can take on a consistent basis to prevent (rather than treat) infection.

Immunoglobulin replacement therapy involves regular infusions that can increase the level of antibodies in your body. This can help to improve your quality of life and prevent infection, as well as related medical complications. 

Immunoglobulin infusions can be delivered intravenously (through an IV) or subcutaneously (via injection). Intravenous Ig treatment (IVIg) is usually given at the hospital, often two to four times per week for two to four hours at a time. IVIG is also given in infusion clinics in outpatient centers set up to deliver this medication. Usually, IVIG is monthly. Subcutaneous Ig treatment (SCIg) is typically delivered at home and may require more frequent doses.

TYPE OF DOCTOR AND DEPARTMENT : Immunologist SPECIALIST CAN DIGNOSES THIS DISEASE.

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