Primary humoral immunodeficiency disease is when a person's immune system is severely weakened, as a result of a lack of B-cells in the blood. A type of white blood cell, B-cells produce antibodies called immunoglobulins which protect and defend against infection. A weakened immune system as a result of a low amount of immunoglobulins is unable to defend against external threats, such as viruses, bacteria, or cancers. This causes infections of all types to develop more easily, occur more frequently, become more severe, and last significantly longer. Primary humoral immunodeficiencies are rare diseases or abnormalities that are either inherited or have been evident since birth.
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Recurrent and severe infections are a common symptom of primary humoral immunodeficiency disease. Infections can occur in various parts of the body, and will recur more often, persist longer, and often lead to further complications if left untreated.
Causes of primary humoral immunodeficiency disease are inherited, and often stem from gene mutations. Primary immunodeficiencies are more commonly found in men, a result of a mutated gene on the X chromosome, which is called an X-linked disorder.
Primary humoral immunodeficiency disease can be diagnosed in a number of ways. A doctor will suspect that a patient has primary humoral immunodeficiency disease if the patient has infections that are frequent, recurrent, and severe. Typically, these types of infections include sinusitis, bronchitis, middle ear infections, and pneumonia. A doctor might also suspect humoral immunodeficiency disease if an infection of a certain cause is significantly more severe than normal.
Primary humoral immunodeficiency disorder is not curable, though early recognition and treatment may greatly prevent infection. Additionally, a number of precautions may be taken by the individual to further prevent infections.
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