Hypergammaglobulinemia is a rare disorder affecting the immune system of the body. It is a condition in which the immune system behaves abnormally to produce increased amount of gamma globulins, a special type of antibodies. Gamma globulins (antibodies) are type of proteins present only in blood.
In hypergammaglobulinemia, an excess amount of antibodies circulate in blood. Among many types of gamma globulin, immunoglobulins are the ones which are checked for diagnosing hypergammaglobulinemia. Patient suffering from this disorder may have enlarged lymph nodes, liver and spleen. They have poor immunity and are prone to opportunistic infections.
Diseases such as HIV, myeloma, hepatitis C, cirrhosis of liver attack the immune system which may result into hypergammaglobulinemia. Treatment consists of immunoglobulin IgG replacement therapy where IgG antibodies are injected for improving immunity and fight infections.
Immunoglobulins are produced by B cells. There are five major classes of immunoglubulins namely IgG, IgM, IgA, IgE, and IgD. In hypergammaglobulinemia, immunoglobulin IgM is majorly found in excess. Normally it is the first immunoglobulin to appear to fight infections. Unfortunately, when IgM is in excess, it in fact weakens the immune system and makes the person susceptible to various infections.
For a person with hypergammaglobulinemia resulting in low immunity even a minor ailment such as common cold can transform into a serious disease such as pneumonia. Persons suffering from this condition often suffer from tuberculosis, frequent episodes of pneumonia, sinusitis, tonsillitis, etc. Patient may also have stiffness of hip joint, fatigue, anemia, lymph node, spleen and liver enlargement.
Types Of Hypergammaglobulinemia
There are five forms of hypergammaglobulinemia. It depends of which immunoglobulin is in excess. Actually different types of antibodies (immunoglobulins ) are produced by class switching of within the B cells. However, in hypergammaglobulinemia, this basic function is imbalanced as a result; any one of the immunoglobulin is produced in excess.
In majority of cases IgM antibodies are produced in excess. It is type 1 or the primary hypergammaglobulinemia. The second type is caused due to mutation of AICDA gene which is found in chromosome 12. The third type is also genetic abnormality, and it is found on the surface of B cell. Type 4 is similar to type 1 but the exact cause is not known. Type 5 is also caused due to mutation of gene called UNG.
Causes Of Hypergammaglobulinemia
Hypergammaglobulinemia is a rare condition. The characteristic of this disorder is that it weakens the immune system. Due to weak immunity, there is very less resistance of the body to fight infection. Several medical conditions are known to cause hypergammaglobulinemia, especially chronic diseases which attack the body for a long period of time.
Diseases such as HIV/ AIDS, Hepatits C, autoimmune disease such as rheumatoid arthritis, myeloma, infectious mononucleosis, viral diseases such as Epstein-Barr disease, POEMS syndrome, cirrhosis of liver etc is known to cause hypergammaglobulinemia.
Treatment For Hypergammaglobulinemia
Hypergammaglobulinemia is an immunoproliferative disorder. In majority of cases if one antibody is in excess there, the other immunoglobulin that may be low. Periodical replacement of this antibody that is low will help in improving the condition.
For example immunoglobulin 1gG constitutes about 75% of the total immunoglobulin. It is found to be low when IgM is high. Because of its low molecular weight, IgG can pass through various compartments and circulate freely in various tissues. IgG plays central role in immunity against bacterial infection. It is the main neutralizing body.
Hence in many cases IgG antibodies are injected to strengthen immunity and fight any infection in people suffering from hypergammaglobulinemia. However, even after treatment, many patients live with low immunity for a long period.