B – cell deficiency

B – cell deficiency 

Introduction:-

Our immune system contain various cells and organs each organs how its own function and for the proper maintenance of body of body each organ should work properly. B &T cells are the main part of lymphoid organs. It the B&T cell are not worked or  there deficiency occurs in our body many infection are get disturbed .The production of antibody is done properly it our B cells are not working properly hence we have so many diseases and disorder due to their deficiency.

B Cell Deficiency:- 

The B cell deficiency cause direct effect on the various immunoglobulin classes there level gets affected due to B cell deficiency sometime we can’t detect there.

Due to B cell deficiency antibody get’s affected and hence their response to common antigen which lacks the antibody deficiency in patients which makes them suffers from phylogenic bacterial infection at four to five months of age. When transferred transplamentally from another disappear. When these antibodies get disappear. This deficiency occurs due to some reasons.

Deficiency:-

1. Failure of differentiation of pre B cells into mature B cells expressing isotopic μ chain on their surface as in Bruton type of agammaglobulinemia that occurs in male infants.
2. Failure of differentiation of mature B cells into plasma cells as in variable type of hypogommaglobulinemia that occur in late adolescence.
3. Low serum IgG levels and decreased number of circulating lymphocytes as is transient hypogamma globulenemia in intancy that makes infants suffer from recurrent that makes infants suffer from recurrent respiratory infection till 3-4 year of age when serum IgG levels and circulating lymphocytes become normal spontaneously.
4. In ability of mature B-cell to makes only one class of Immunoglobulin that occurs in selective antibody deficiency such as IgA antibody deficiency related disorder or disease:-
5. Bruton’s  X- linked hypogammaglobulinemia :-
6. It is an X-linked hypogammaglobulinemia several symptoms or effective are seen during it’s this deficiency.
7. Serum IgG molecules are undetectable.
8. B- cells in circulation and antibody response to vaccines are absent.
9. Lymphoid tissue is hypoplastic and B–cell dependent areas are poorly developed.
10. Plasma cell cannot be seen.

Selective type of immunoglobulin deficiency for ex:- IgA deficiency to allowing symptoms one effects are found .It is the most deficiency compound to among all deficiency of IgG or IgM deficiency

In Hypogammaglobulinemia:- 

• This is variable type of hypogammaglobulinemia occurs in late adolensence antibody may be due to the ether excessive number of suppresser T – cells as a patient with thymoma or due to abnormalities of helper t- cells as in selective  IgM  deficiency. 
• In this T – cell function is usually normal. As a result cell mediated immunity remain effective and there by affected infant can resist infection with viruses.
• Due to the antibody deficiency autoimmune disorder or polyarthritis are very common in patient with antibody deficiency.

Treatment :- 

Treatment of B -cell or antibody deficiency depends on types of deficiency .

Disorder:- 

(Bruton’s X – linked hypogammaglobulinemia)

B-Cell Immunodeficiency :-

Hypogammaglobulinemia is present in up to 85% of CLL patients and like is a type of primary immuno deficiency  disease in which not enough gamma globulins exist in the blood 

This entails that not enough antibodies exist which impairs the immune system. Hypogammaglobulemia is a characteristics of common variable immunodeficiency. Other causes include loss of gamma globulins in urines as in nonselective glomerular proteinuria.

Diagnosis :- Treatment :-

Treatment is by parental  administration of gamma globulins either monthly intravenously or more recently by weekly self administend hypodermolysis .

In either case mild allergic reaction (generalized pruritus ,urticaria ) are common and are usually manageable with oral diphenylhydramine.