Hypogammaglobulinemia: What is it? Types Causes, Symptoms, Diagnosis and Treatment

A reduction in the amount of antibodies in the body is linked to periodic infections by particular forms of bacteria.

Hypogammaglobulinemia is a medical complication caused by a deficiency of B cells (B lymphocytes) in addition to a resulting decrease in the amount of antibodies ( immunoglobulin ) in the body system.

These antibodies play a double role in the body’s immune system by allowing foreign antigens in addition to generating a genetic response that ultimately results in the eradication of these antigens.

A 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.

Also known as common variable immunodeficiency, agammaglobulinemia (X-linked), IgA deficiency and transient hypogammaglobulinemia of childhood.

Antibodies are key 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 amount of antibodies leads to hypogammaglobulinemia.

Lymphocytes:

While T lymphocytes are responsible for the destruction of 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

Hipogammaglobulinemia city:

Primary hypogammaglobulinemia occurs due to damage to B cells. This may be as 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.

Secondary Hypogammaglobulinemia:

The secondary hypogammaglobulinemia is caused by a disorder that indirectly hinders the manufacture or 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.

Transient hypogammaglobulinemia:

Occasionally, newborn babies suffer a decrease in the amount of antibodies since their respective immune systems are not mature enough to make antibodies.

Subsequently, newborn babies depend on the amount 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 main causes of hypogammaglobulinemia. It produces a decrease in the level of immunoglobulins IgM, IgA and IgG in the body system.

It is a deficiency in the immune system that is inherited ( congenital hypogammaglobulinemia ) or acquired. In most scenarios, the family medical history does not matter.

However, in scenarios where two or more family members are diagnosed with the condition, a type of atypical gene inheritance may be a possibility.

In approximately 5 percent of the scenarios, it is said that at least one member of the family 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.

Periodic infections can affect up to half of the patents suffering from IgA deficiency disorder. However, most people affected may not have any health problems.

Several patients experience symptoms of hypogammaglobulinemia after having a common 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 is a hereditary disease 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 pneumoniae, Haemophilus influenzae and Staphylococcus. They can also be attacked by frequent viral infections.

Examples of the most affected regions include the skin, lungs, respiratory tract and gastrointestinal tract.

People who experience this complication may have a family history characterized by immune disorders such as agammaglobulinemia.

symptom

Some of the frequent infections that are experienced include:

  • Respiratory tract infections.
  • Infections of the skin.
  • Sinusitis.
  • Pneumonic infections
  • Middle ear infections (otitis media).
  • Eye infection ( conjunctivitis ).
  • Diarrhea.
  • Bronchitis.

Normally, these infections affect people before they turn 4 years old.

The additional infections are a mysterious asthma in addition to a bronchiectasis , which is a disorder characterized by the destruction and expansion of small air sacs found in the lungs.

Bruton’s disease is a condition whose symptoms are evidenced between the age of 7 and 9 months due to a significant decrease in the level of 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.

Causes

As is already known, hypogammaglobulinemia is the result of a decrease in the production of white blood cells and immunoglobulins in the body system. It can also be caused by secondary factors.

Secondary factors that cause hypogammaglobulinemia should be evaluated before a patient can be diagnosed with the condition.

The secondary hypogammaglobulinemia can also be a result of the decrease in proteins produced by enteropathy.

This condition is related to a reduction in the level of albumin plus imprecise loss of serum protein. The type T of cells can also be reduced due to enteropathies.

Diagnosis

The diagnosis begins with a physical test and an evaluation of the patient’s family medical history. Normally, physical evaluation is standard for individuals who have agammaglobulinemia.

The test is an important aspect of the diagnosis of hypogammaglobulinemia.

Some of the tests that are 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 takes into account the fact 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 as well as the risks of long-lasting lung infections.

The test indicates that there is a relationship between large amounts of IgG and a lower frequency of infections.

The results are pitiful in the event that hypogammaglobulinemia is not discovered and treated, especially in cases of bronchiectasis or severe destruction of the lungs.

Unfortunately, the diagnosis ends up being too late, which increases the risks of a series of pulmonary infections and 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, both with different survival rates.

The information indicates a survival rate of 78 percent over a period of 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 is 97 percent.

In the same period of time. Therapy with the use of immunoglobulin increases the chances of survival, especially when the diagnosis of hypogammaglobulinemia is made at an early stage.

People with this condition and who need immunoglobulin therapy should be closely monitored by competent and highly qualified medical personnel to reduce the risks of malignancy and other infections.

Treatment

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, there are a number of treatments that can be used to restore or increase the amounts of antibodies.

Hypogammaglobulinemia is often controlled by the use of 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 a wide variety of antibiotics.

People who experience bronchiectasis may require a continuous physical treatment, as well as 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, among others.

People with arthritis or an immunodeficiency can receive enough doses of gamma globulin to get enough relief from the symptoms.

It is important to keep in mind that early and complete therapy with the use of antibiotics helps fight various bacterial infections.

It also helps reduce the risks of severe infections. Individuals who have agammaglobulinemia should not receive common vaccines for the control of 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 members of the family experienced preimplantation as a result of their parents’ inheritance of the disease.

Prevention involves monitoring patients carefully to detect any signs of secondary hypogammaglobulinemia.

Important information

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 so they can begin using them immediately, as they detect an infection or discomfort.

It is important to avoid taking live vaccines in general.

Guidance should be provided regarding travel abroad to obtain inoculation, use of antibiotics while traveling, take only boiled or safe water.

Prepare adequate medical insurance before travel and ensure that immunoglobulin therapy continues as needed.