A reduction in the number of antibodies in the body is linked to recurring infections by particular forms of bacteria.
Hypogammaglobulinemia is a medical complication caused by a deficiency of B cells (B lymphocytes) and a decreasing number of antibodies ( immunoglobulin ) in the body system.
These antibodies play a double role in the body’s immune system by allowing foreign antigens and generating a genetic response that ultimately results in eradicating these antigens.
In the case of pure B lymphocyte disorder, the state of immunity in the cells is integral, and the recurrence of bacterial, fungal, and viral infections does not increase.
The most frequently experienced congenital complications of B cells are Hypogammaglobulinemia.
They are also known as common variable immunodeficiency, agammaglobulinemia (X-linked), IgA deficiency, and transient Hypogammaglobulinemia of childhood.
Antibodies are vital proteins responsible for labeling pathogens that cause disease for damage.
The decrease in the level of antibodies in the body leaves a person exposed to the attack of infections. The decrease in the number of antibodies leads to Hypogammaglobulinemia.
While T lymphocytes are responsible for destroying disease-causing organisms, B lymphocytes make specific antibodies that attach to the attacking organism, suggesting that T lymphocytes and other defense cells group and attack these foreign organisms.
Types of Hypogammaglobulinemia
Primary Hypogammaglobulinemia occurs due to damage to B cells. This may be a result of cancer chemotherapy.
It should be clear that cancer directly damages the bone marrow, which is the place where B cells are produced. It can also be caused by a genetic complication that disables all the antibodies produced.
The secondary Hypogammaglobulinemia is caused by a disorder that indirectly hinders the manufacture of action of the antibodies.
This can be a very unbearable condition since it can lead to the depletion of all the amounts of the remaining antibodies.
Occasionally, newborn babies decrease the number of antibodies since their respective immune systems are not mature enough to make antibodies.
Subsequently, newborns depend on the number of antibodies transferred to them from their respective mothers during pregnancy or through the diet after birth.
The common variable immunodeficiency is one of the leading causes of Hypogammaglobulinemia. It decreases immunoglobulins IgM, IgA, and IgG levels in the body.
It is a deficiency in the immune system inherited ( congenital Hypogammaglobulinemia) or acquired. In most scenarios, the family medical history does not matter.
However, a type of atypical gene inheritance may be possible in scenarios where two or more family members are diagnosed with the condition.
In approximately 5 percent of the scenarios, it is said that at least one family member lacks IgA immunoglobulin.
Immunoglobulin IgA type deficiency is the most widespread of all deficiency disorders affecting antibodies. However, it is evident in approximately 1 in every 700 people.
Frequent infections can affect up to half of the patients suffering from IgA deficiency disorder. However, most people affected may not have any health problems.
Several patients experience symptoms of Hypogammaglobulinemia after having a typical childhood. Infections of the gastrointestinal tract and the lower and upper sections of the respiratory tract are not uncommon.
People with imperceptible amounts of IgA immunoglobulin are at risk of severe hypersensitivity if they receive blood or their products.
Hypogammaglobulinemia linked to the X chromosome:
This hereditary disease is characterized by low amounts of defensive immune system proteins known as immunoglobulins. People who experience this disease often suffer from various infections.
While both sexes may experience this condition, men are at greater risk. It is caused by atypical genes that hinder the growth of healthy B cells.
Subsequently, the immune system receives low amounts of immunoglobulins. Immunoglobulins are responsible for protecting the body against infections and diseases.
The lack of defensive immunoglobulins leads to frequent infections and diseases.
People affected by this condition are especially vulnerable to attack by bacteria such as Streptococcus pneumonia, Haemophilus influenzae, and Staphylococcus. Frequent viral infections can also attack them.
The most affected regions include the skin, lungs, respiratory tract, and gastrointestinal tract.
People who experience this complication may have a family history of immune disorders such as agammaglobulinemia.
Some of the frequent infections that are experienced include:
- Respiratory tract infections.
- Infections of the skin.
- Pneumonic infections
- Middle ear infections (otitis media).
- Eye infection ( conjunctivitis ).
Usually, these infections affect people before they turn four years old.
The other infections are mysterious asthma in addition to bronchiectasis, which is a disorder characterized by the destruction and expansion of tiny air sacs found in the lungs.
Bruton’s disease is a condition whose symptoms are evidenced between the ages of 7 and 9 due to a significant decrease in maternal antibodies.
The condition that causes frequent infections by bacteria affects only men. Some of the frequent infections include pneumonia, sinusitis, and otitis media.
Frequently, patients may also experience weak or small lymph nodes and tonsils. About fifteen percent of these patients suffer death due to infections before they reach the age of 20 years.
As is already known, Hypogammaglobulinemia is the result of a decrease in the production of white blood cells and immunoglobulins in the body system. Secondary factors can also cause it.
Secondary factors that cause Hypogammaglobulinemia should be evaluated before a patient can be diagnosed.
The secondary Hypogammaglobulinemia can also result from the decrease in proteins produced by enteropathy.
This condition is related to a reduction in albumin level plus imprecise loss of serum protein. The type T of cells can also be reduced due to enteropathies.
The diagnosis begins with a physical test and an evaluation of the patient’s family medical history. Usually, a physical evaluation is standard for individuals who have agammaglobulinemia.
The test is an essential aspect of the diagnosis of Hypogammaglobulinemia.
Some of the tests carried out during the evaluation of agammaglobulinemia are the serum IgM test, the serum IgA test and the serum IgG test, and serum immunoelectrophoresis.
Some of the tests used in the diagnosis of Hypogammaglobulinemia are:
- Low-level serum B cells and immunoglobulins.
- Shortage of particular antibodies in all vaccines administered to the child.
- Deficiency of particular antibodies in blood group A and antigens of blood group B.
Prognosis de Hipogammaglobulinemia
In general, it is difficult to oversimplify the prognosis of Hypogammaglobulinemia because there are possibly other underlying complications.
This considers that the prognosis of Hypogammaglobulinemia is determined by the results of all the underlying complications.
However, early diagnosis and treatment of Hypogammaglobulinemia may not help minimize morbidity rates and the risks of long-lasting lung infections.
The test indicates a relationship between large amounts of IgG and a lower frequency of infections.
The results are pitiful if Hypogammaglobulinemia is not discovered and treated, especially in bronchiectasis or severe destruction of the lungs.
Unfortunately, the diagnosis is too late, increasing the risk of many pulmonary infections. This is attributed to the reduction in the life expectancy of Hypogammaglobulinemia.
There is little information about mortality rates. The information indicates unique medical phenotypes of unpredictable mutual immunodeficiency, with different survival rates.
The information indicates a survival rate of 78 percent over ten years during treatment with immunoglobulin.
Previous studies indicated that the survival rate remained at 37 percent during the same period when low immunoglobulin therapy was used in a population where the ordinary survival rate was 97 percent.
In the same period. Therapy with immunoglobulin increases the chances of survival, especially when the diagnosis of Hypogammaglobulinemia is early.
To reduce the risks of malignancy and other infections, people with this condition who need immunoglobulin therapy should be closely monitored by competent and highly qualified medical personnel.
Before starting treatment, the doctor should consider the dangers and benefits of administering any medication that can reduce the level of antibodies in the patient’s bloodstream.
While the hereditary causes of this condition are impossible to avoid, several treatments can be used to restore or increase the amounts of antibodies.
Hypogammaglobulinemia is often controlled by IVIG (intravenous gamma globulin). This should be administered after 21 or 28 days subcutaneously or intravenously.
Treatment with antimicrobial Hypogammaglobulinemia should be initiated immediately when an infection is detected.
People with a severe lung disorder or sinusitis may require continuous therapy with various antibiotics.
People who experience bronchiectasis may require continuous physical treatment and the elimination of pus and secretions from the lungs daily.
People suffering from malabsorption or gastrointestinal complications should undergo tests for infections such as rotavirus and Giardia lamblia.
People with arthritis or immunodeficiency can receive enough doses of gamma globulin to get enough relief from the symptoms.
It is essential to remember that early and complete therapy with antibiotics helps fight various bacterial infections.
It also helps reduce the risks of severe infections. Individuals with agammaglobulinemia should not receive common vaccines to control typical viral infections such as rubella, mumps, and measles.
This is because they can cause a secondary infection.
Secondary control of Hypogammaglobulinemia
Family members of the patient with Hypogammaglobulinemia who have recognized inherited mutations responsible for the disease can receive relevant counseling services before a negotiable examination of the genes.
Several cases were identified in which the other family members experienced preimplantation due to their parents’ inheritance of the disease.
Prevention involves monitoring patients carefully to detect any signs of secondary Hypogammaglobulinemia.
Patents are highly advised to seek medical help for any case of infection or discomfort as soon as possible.
In some cases, health care providers may recommend that patients keep recommended antibiotics near them at home to begin using them immediately, as they detect infection or discomfort.
It is essential to avoid taking live vaccines in general.
Guidance should be provided regarding travel abroad to obtain inoculation, use antibiotics while traveling, and take only boiled or safe water.