Hypogammaglobulinemia

What is Hypogammaglobulinemia?

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

These antibodies play a twofold part in the body’s immune system by allowing alien antigens besides generating a genetic response that ultimately results in the eradication of those antigens. A reduction in the amount of antibodies in the body is linked to periodic infections by particular forms of bacteria. A case of pure B-lymphocyte disorder, the immunity state in the cells is integral and recurrence of bacterial, fungal and viral infections does not rise up.

The most frequently experienced congenital complications of the B-cells are Hypogammaglobulinemia, (also referred to as Common Variable Immunodeficiency or common variable hypogammaglobulinemia) Agammaglobulinemia (X- linked), IgA Deficiency and Transient Hypogammaglobulinemia of infancy.

Antibodies are key proteins responsible for tagging disease-causing pathogens for damage. Decrease in the level of antibodies in the body leaves a person exposed to attack by infections. Decrease in the amount of antibodies leads to Hypogammaglobulinemia.

Generally, there are 2 kinds of lymphocytes. While T-lymphocytes are responsible for the destruction of the disease-causing organisms, B-lymphocytes manufacture the specific antibodies that cling on the attacking organism, thereby hinting the T-lymphocytes as well as other defense cells to group and attack those foreign organisms.

Primary Hypogammaglobulinemia

Primary hypogammaglobulinemia is brought about by damage to the B-cells. This may be as a result of cancer chemotherapy. It is should be clear that cancer directly damages the bone marrow, which is the location for the production of B-cells. It may also be brought about by a genetic complication that renders all the produced antibodies useless.

Secondary Hypogammaglobulinemia

Primary hypogammaglobulinemia is brought about by a disorder that indirectly hinders the manufacture or action of the antibodies. This can be a very excruciating condition because it can lead to the depletion of all amounts of the remaining antibodies.

Transient hypogammaglobulinemia

Occasionally, newly born babies suffer from a decrease in the amount of antibodies since their respective immune systems are not sufficiently mature to manufacture antibodies. Subsequently, newly born babies depend on the amount of antibodies transferred to them from their respective mothers during the pregnancy or via the diet after birth.

Common Variable Immunodeficiency is one of the key causes of Hypogammaglobulinemia. It results in a decrease in the level of IgM, IgA, and IgG kinds of immunoglobulins in the body system. It is a deficiency in the immune system which is either inherited (congenital hypogammaglobulinemia) or acquired ( acquired hypogammaglobulinemia) . In most scenarios, the family medical history does not matter. Nevertheless, in scenarios where two or more members of the family are diagnosed with the condition, a two atypical gene type of inheritance can be a possibility. In approximately 5 percent of the scenarios, at least one member of the family is said to lack the IgA immunoglobulin.

Deficiency of the IgA type of immunoglobulin is the most widespread of all deficiency disorders that affect the antibodies. However, it is evidenced in roughly 1 person out of 700. Periodic infections can affect up to half of patents suffering from IgA deficiency disorder. However, majority of the affected persons may not be in any health problem. A number of patients experience symptoms of hypogammaglobulinemia after having an ordinary infancy. Infections of the gastrointestinal tract and the lower and upper sections of the respiratory tract are not uncommon. Individuals with imperceptible amounts of IgA immunoglobulin risk intense hypersensitivity in case they are given any blood or its products.

Agammaglobulinemia (X- linked hypogammaglobulinemia)

This is a hereditary disease characterized by low amounts of defensive proteins of the immune system known as immunoglobulins. Individuals experiencing this disease frequently suffer various infections.

While both sexes can experience this condition, the males are at a higher risk. It is brought about by atypical genes that hinder the growth of healthy B-cells. Subsequently, the immune system receives low amounts of immunoglobulins. Immunoglobulins are responsible for the protection of the body against infections and illness.

Lack of the defensive immunoglobulins leads to frequent infections and illnesses. Individuals affected by this condition are specifically vulnerable to attack by bacteria like Streptococcus pneumoniae, Haemophilus influenzae as well as staphylococci. They may also be attacked by frequent viral infections. Examples of the most affected regions include the skin, lungs, respiratory tract and gastrointestinal tract. Individuals experiencing this complication possibly have a family past characterized by immune disorders like Agammaglobulinemia.

Some of the frequent infections that are experienced include:

  • Infections of the respiratory tract
  • Infections of the skin
  • Sinusitis
  • Pneumonic infections
  • Infections of the middle ear (otitis media)
  • Infection of the eye (conjunctivitis)
  • Diarrhea
  • Bronchitis

Normally, these infections affect individuals before they reach the age of 4. Additional infections are a mysterious asthma as well as bronchiectasis, which is a disorder that is characterized by destruction and expansion of the tiny air sacs found in the lungs.

Bruton 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 motherly antibodies. The condition which results in frequent infections by bacteria affects males only. Some of the frequently experienced infections include pneumonia, sinusitis, and otitis media. Frequently, the patients may also experience feeble or little lymph nodes and tonsils. About fifteen percent of these patients suffer death due to infections before they reach the age of 20.

Causes of hypogammaglobulinemia

As you are already aware, hypogammaglobulinemia results from a decrease in the manufacture of white blood cells and immunoglobulins in the body system. Its can also be caused by secondary factors.

Secondary factors that cause hypogammaglobulinemia must be evaluated before a patient can be diagnosed as having the condition.

Secondary hypogammaglobulinemia can also be a result of decrease in proteins brought about by enteropathy. This condition is linked to a reduction in the level of albumin plus imprecise loss of serum protein.

T-type of cells may also reduce due to enteropathies.

Hypogammaglobulinemia diagnosis

Diagnosis of hypogammaglobulinemia starts with a physical test and evaluation of the patient’s family medical history. Normally, the physical assessment is standard for individuals having agammaglobulinemia. Examination is an important aspect of the diagnosis of hypogammaglobulinemia. Some of the tests that are carried out during the assessment of agammaglobulinemia are serum IgM test, serum IgA test, and serum IgG test and serum immunoelectrophoresis.

A few of the tests that are used in the diagnosis of hypogammaglobulinemia are:

  1. Low level serum b-cells and immunoglobulins
  2. Shortage of particular antibodies in all vaccinations administered to the child
  3. Deficiency of particular antibodies in blood group A and blood group B antigens.

Hypogammaglobulinemia prognosis

It is generally hard to oversimplify the prognosis of hypogammaglobulinemia because possibly there are a number of other underlying complications. This takes into account the fact that hypogammaglobulinemia prognosis is determined by the results of all underlying complications. However, early diagnosis and treatment for hypogammaglobulinemia may not help to minimize the rates of morbidity as well as the risks for lasting pulmonary infections. Proof indicates that there is a relationship between large amounts of IgG and decreased frequency of infections.

Results are pitiable in case hypogammaglobulinemia is not discovered and treated, especially in cases of a bronchiectasis or a severe destruction of the lungs. Sadly, the diagnosis ends up being overly late, thereby increasing the risks for a number of pulmonary infections and this attributes to reduced life expectancy of hypogammaglobulinemia.

There’s little information on mortality rates. Information states unique medical phenotypes of mutual unpredictable immunodeficiency, both having distinct rates of survival. Information indicates a 78 percent rate of survival over a ten-year period while on immunoglobulin therapy. Previous studies indicated that the rate of survival stood at 37 percent over the same tenure when low immunoglobulin therapy was used in a population where the ordinary survival rate is 97 percent over the same period of time. Therapy with the use of immunoglobulin increases the chances of survival, especially when the diagnosis of hypogammaglobulinemia is done at an early stage.

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

Treatment of hypogammaglobulinemia

Before the start of hypogammaglobulinemia treatment, the health care provider should consider the dangers and advantages of administering any medications that can reduce the level of antibodies in the patient’s bloodstream.

While hereditary causes of this condition are impossible to avoid, there are a number of treatments that can be used to restore or enhance the amounts of antibodies. Hypogammaglobulinemia is often controlled through the use of IVIG (Intravenous Gamma globulin). This should be administered after 21 or 28 days subcutaneously or intravenously.

Antimicrobial hypogammaglobulinemia treatment must be started immediately an infection is detected. Individuals suffering from lung disorder or severe sinusitis may require continuous therapy with a wide range of antibiotics. Individuals experiencing bronchiectasis may require continuous physical treatment as well as removal of pus and discharges from the lungs on a daily basis. Individuals suffering from malabsorption or gastrointestinal complications should undergo examinations for infections like rotavirus and Giardia lamblia among others. Individuals with arthritis or an immuno deficiency can be given sufficient doses of gammaglobulin so as to get enough comfort from the symptoms.

It is important to note that early and thorough therapy with the use of antibiotics helps to fight various bacterial infections. It also helps to reduce the risks of intense infections. Individuals having agammaglobulinemia must not be given common vaccines for control of typical viral infections like rubella, mumps and measles. This is because they may result in a secondary infection.

Screening

Screening of people without the hypogammaglobulinemia symptoms is not specified. In particular cases, members of the family may undergo screening but for precise requirements only.

Secondary control of hypogammaglobulinemia

Family members of the hypogammaglobulinemia patient having recognized hereditary mutations responsible for the condition can be provided with relevant counseling services ahead of a negotiable examination for genes. A number of cases have been identified where the other family members experience preimplantation as a result of inheritance of the condition from their parents. Prevention entails monitoring the patients carefully for any signs of secondary hypogammaglobulinemia.

Important information

Patents are highly warned to look for medical help for any cases of infection or discomfort as soon as possible. In a few cases, health care providers may recommend that patients keep the recommended antibiotics near them at home so that they can start using them immediately they detect an infection or discomfort.

It is important to avoid taking live vaccines all in all. Guidance concerning travels abroad should be provided as far as getting inoculation, using antibiotics while traveling, taking boiled or safe water only, preparing suitable medical insurance ahead of the trip and making sure that the immunoglobulin therapy is continued as necessary.