Hypogammaglobulinemia is a clinical picture that belongs to the group of so-called primary immunodeficiencies. It is characteristic of this special immune defect that it is determined by a lack of antibodies. Basically, hypogammaglobulinemias are diseases in which gamma globulins, especially immunoglobulins, are completely absent.
What is hypogammaglobulinemia?
Conjunctivitis is increasing in the eyes, while purulent sniffles appear in the nose. See AbbreviationFinder for abbreviations related to Hypogammaglobulinemia.
The term hypogammaglobulinemia is mostly used as a synonym for the term agammaglobulinemia. The only difference is that in hypogammaglobulinemia the gamma fraction is reduced, while in agammaglobulinemia it does not exist at all.
Observations have shown that a large proportion of agammaglobulinemias are essentially hypoglobulinemias. In most cases, however, this difference is not of clinical relevance. On the other hand, hypogammaglobulinemia is differentiated from dysgammaglobulinemia, which is far more common.
Both hypogammaglobulinemia and dysgammaglobulinemia occur in a variety of immunodeficiencies, such as WHIM syndrome, ICOS deficiency, CVID, and hyper-IgM syndrome. Hypogammaglobulinemia is also possible as an accompanying symptom. In general, antibody deficiency diseases such as hypogammaglobulinemia are the most common immune defects.
Causes
In principle, all antibody deficiency diseases, including hypogammaglobulinemia, are based on a reduced production of antibodies. Antibodies are medically called immunoglobulins. These are endogenous proteins that are used to defend against special microorganisms.
Basically, a distinction is made between congenital, so-called primary hypogammaglobulinemia and, on the other hand, acquired or secondary hypogammaglobulinemia. Secondary hypogammaglobulinemia can be caused by certain diseases, such as malignant or malignant diseases. These are often related to the system responsible for blood formation.
For physiological reasons, hypogammaglobulinemia occurs in babies between the ages of two and six months. During this period of time, the infant’s organism replaces the antibodies that were absorbed by the mother in the uterus with self-produced ones. During this period, the probability of serious bacterial infections is increased.
A special and also relatively common form of hypogammaglobulinemia is the so-called selective IgA deficiency. In some antibody deficiency diseases, such as agammaglobulinemia, genetic causes are also involved in the development of the disease.
Symptoms, Ailments & Signs
In the context of hypogammaglobulinemia, the affected patients can develop a large number of different symptoms and complaints. A feared complication when carrying out vaccinations is a lack of reaction to the vaccine antigens.
In addition, in many cases, the disease leads to an increased susceptibility to bacterial infections. The resulting infections often affect the upper respiratory tract, the gastrointestinal tract and the skin. In addition, hypogammaglobulinemia is a major feature of a number of syndromes.
Mental retardation is sometimes associated with X-linked hypogammaglobulinemia and neurological deficits. X-linked hypogammaglobulinemia also occurs in some cases of short stature resulting from an isolated lack of growth hormones. In addition, the disease is part of the osteopetrosis-hypogammaglobulinemia syndrome.
Basically, antibody deficiency diseases, which include hypogammaglobulinemia, differ based on the severity and severity of the symptoms. The diseases usually have in common that the patients suffer from recurring infections. These primarily affect the paranasal sinuses and ears, for example in the form of a middle ear infection.
Conjunctivitis is increasing in the eyes, while purulent sniffles appear in the nose. The bronchi are often affected by bronchitis, and pneumonia can occur in the lungs. Recurring infections of the lungs can lead to chronic changes in the organ.
The bronchi dilate, making it easier for pus to accumulate. Diarrhea is favored by frequent infections of the gastrointestinal tract. Autoimmune reactions are also possible, in which the immune system attacks endogenous substances and tissues.
Diagnosis & course of disease
In order to diagnose hypogammaglobulinemia, it is essential for the affected person to consult an appropriate medical specialist. He first discusses the medical history, possible previous illnesses and the individual lifestyle with the patient. The symptoms described are of central importance, since they provide information about the severity of the hypogammaglobulinemia.
Blood tests are necessary to diagnose an antibody deficiency with certainty. Antibodies are detectable in the blood and can be precisely quantified. In addition, the deficiency of a single subgroup of antibodies can be precisely determined. An analysis of so-called specific antibodies, which are directed against special pathogens and components of blood groups, must also be carried out.
Complications
As a result of hypogammaglobulinemia, the affected person is usually ill relatively often and often affected by infections and inflammations. This mainly leads to infections of the respiratory tract, resulting in breathing difficulties or pneumonia. In the worst case, this can be fatal for the patient.
Stomach problems can also occur. In many cases, the patients suffer from reduced intelligence and retardation, so that they are dependent on the help of other people in everyday life. Not infrequently, hypogammaglobulinemia also leads to short stature. As the disease progresses, inflammation also occurs in the nasal cavities or in the middle ear.
The constant infections and inflammations can also have a negative effect on the patient’s mental state and lead to a reduced quality of life. Stomach problems often lead to diarrhea or abdominal pain. As a rule, it is not possible to permanently cure hypogammaglobulinemia.
The treatment is therefore carried out with the help of infusions and medication and can limit the symptoms for a short period of time. However, the person concerned has to undergo the treatment several times. Life expectancy is usually reduced by hypogammaglobulinemia.
When should you go to the doctor?
Since hypogammaglobulinemia does not heal itself and in most cases also leads to a negative course of the disease, the affected person must consult a doctor. This can prevent further complications and discomfort. A doctor’s visit is necessary if the patient frequently suffers from infections and inflammations.
The respiratory tract is particularly affected by these infections, which can lead to severe breathing difficulties. Mental retardation is also often an indication of hypogammaglobulinemia and should be examined by a doctor. Discomfort in the eyes or frequent inflammation of the conjunctiva can also indicate the disease. The sooner the complaints are examined, the higher the probability of a complete cure.
In most cases, hypogammaglobulinemia can be diagnosed by a general practitioner or pediatrician. In the further treatment, however, the involvement of different specialists is necessary to alleviate the symptoms of hypogammaglobulinemia. If diagnosed early, there is usually no reduction in life expectancy.
Treatment & Therapy
So far, there are no known ways to cure an antibody deficiency in general and hypogammaglobulinemia in particular in the long term. However, affected patients can receive a substitute for the immunoglobulin G. This is the main component of antibodies. For this purpose, special antibodies are filtered out of the blood of plasma donors.
After undergoing extensive purification, the antibodies are administered to subjects either intravenously or subcutaneously by periodic injection. By supplying antibodies in this way, recurring infections can be contained well in most cases.
Prevention
Concrete measures to prevent hypogammaglobulinemia are not yet known. In addition, the disease also has a genetic component that cannot be influenced. It is all the more important to consult a doctor immediately if signs or symptoms of the disease appear.
Aftercare
In the case of hypogammaglobulinemia, in most cases the affected person has no special options for aftercare. Those affected should contact a doctor as soon as the first symptoms and signs of the disease appear so that treatment can be initiated at an early stage. Complete healing is not possible.
If there is a desire to have children, a genetic examination and counseling can also be carried out in the case of hypogammaglobulinemia. This may prevent the disease from recurring in offspring. Those affected are usually dependent on taking various medications. It is always important to follow the doctor’s instructions, whereby the correct dosage and regular intake should be observed.
If anything is unclear or if you have any questions, you should always consult your doctor first. Since a complete cure of hypogammaglobulinemia is not possible, those affected are dependent on taking the medication for life. Further follow-up measures are usually not necessary. The disease also does not reduce the life expectancy of the affected person. However, the risk of infectious diseases is relatively high due to the disease, so that those affected should take special protection against them.
You can do that yourself
Since the lack of immunoglobulins accounts for the condition of hypogammaglobulinemia, self-help is primarily about strengthening and supporting your own immune system.
The risk of easily contracting infectious diseases is difficult for many patients to handle. But there are some precautions to take, the challenge is to avoid bacteria as much as possible. Hand cleaners, which are available in every drugstore, are suitable for this. A large part of the immune system resides in the gut, or depends on gut health. Probiotics and a healthy diet help to keep the intestines fit. Fruit and vegetables, especially salads, should be washed thoroughly. Wherever bacteria can grow, cleaning and washing is very important for hypogammaglobulinemia patients.
Participating in support groups is helpful for many people who have hypogammaglobulinemia. Especially in the time after the diagnosis, the emotional support that is offered in groups is a great support. In the exchange of experiences, the individual measures to increase the quality of life and to combat hypogammaglobulinemia are exchanged. But patients and relatives also come together on online forums to exchange experiences and have the opportunity to alleviate individual symptoms with the support of others.
