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Hypogammaglobulinemia

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Acute

What is Hypogammaglobulinemia?

Primary (congenital) hypogammaglobulinemia: Several rare genetic disorders called primary immunodeficiency disease (PIDD) cause low immunoglobulin. With PIDD, you're born with a mutation, or error, in the genetic material that tells your immune system how to work. These mutations often run in biological families

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  • Ear infections (ear pain, scarred eardrum, fever).
  • Sinus infections (runny or stuffy nose, fatigue, fever).
  • Bronchitis (persistent cough, shortness of breath, fever).

Without treatment, hypogammaglobulinemia can cause severe infections with potentially life-threatening complications. One of the most common complications is bronchiectasis. This condition involves irreversible lung damage. It's common among people with frequent upper respiratory infections.

CVID is the most common cause of primary hypogammaglobulinemia in adults. X-linked agammaglobulinemia (XLA): A mutation on the X chromosome prevents your body from making enough B-cells or immunoglobulins. XLA primarily affects babies assigned male at birth (as they only have one X chromosome).

B-cell depletion induced by rituximab (RTX) in ANCA-associated vasculitis (AAV) is a risk factor for hypogammaglobulinemia. Aggregating data on gammaglobulin levels kinetics during RTX and its association with the risk of relapse and severe infection is of interest.

People who get severe or frequent bacterial infections may need to take antibiotics for several months at a time to prevent them. If your hypogammaglobulinemia is severe, you may get immune globulin replacement therapy to replace what your body isn't making.

How is it diagnosed?

How is it diagnosed?

Treatment is tailored to specific causes and the clinical presentation of each patient, and includes intravenous or subcutaneous immunoglobulin replacement. Severe combined immunodeficiency is a medical emergency and suspected cases require immediate specialist centre referral for diagnosis and treatment.

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How is it treated?

Treatment for acute myeloid leukemia is vital. It varies with the patient and stage of the disease. Treatment options include

  • Your symptoms. Give your care team a detailed history of your or your child's symptoms — which foods, and how much, seem to cause problems.
  • Your family history of allergies. ...
  • A physical examination. ...
  • A skin test. ...
  • A blood test. ...
  • Elimination diet. ...
  • Oral food challenge.

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