ABNORMALITY AT THE IMMUNOLOGIC LEVEL OF

PANHYPOGAMMAGLOBULINEMIA
Panhypogammaglobulinema this is associated having low level of immunoglobulins.
Immunoglobulins are very important in the body for it presents the serum and cells of the
immune system. Its main function is the production of antibodies. Antibodies are
immunoglobulins that is responsible for removal and specification of a foreign antigen which is
harmful to the human body.
The main cause of the absence or loss of an antibody is due to the plasma cells that have
abnormalities in the development of B lymphocytes.
Other than the cause of B cells, scientist found out that T cells can either contribute to the
abnormalities of producing antibody which protects the body from infections. The lack of T cell
maturation have an effect on the development of B cell that afterwards B cells have poor
development.
Some patients having panhypogammaglobulinema can accumulate abnormality in the lymphatic
system especially in the lymph nodes. They are also susceptible to gastrointestinal complications,
and have a high potential for having cancer. Some may also have complications on developing
granular, inflammatory nodules in the skin, spleen, liver, and lungs.
Genetics have also an impact in getting this disorder. The abnormal gene can be inherited from
parent which results into new mutation (gene change) in the affected individual.
The determination in the immunologic abnormality of this disease shows a significant role in the
diagnosis of pahypogammaglobulinema. The diagnosis of this disease is primarily established by
testing for low blood (serum) IgG immunoglobulin concentrations ranging from severely
reduced (<100 mg/dL) to just below adult normal range (500-1200 mg/dL). In addition,
laboratory testing may reveal normal or, in some cases, reduced numbers of circulating B cells.
Failure of certain B cells to appropriately mature into antibody-producing plasma cells may also
be detected. Specialized laboratory tests may also help to determine the exact nature of the
immune defect (e.g., B cell, helper T cell, suppressor T cell, or B and T cell defects).(1)

REFERENCES:
(1)National Organization for Rare Disorders. (2014). Common Variable Immune Deficiency. 5
Kenosia Ave., Danbury CT 06810. Retrieved from: https://rarediseases.org/rarediseases/common-variable-immune-deficiency/