Buon's agammaglobulinemia is due to?
Ans is 'a' i.e. B-cell defect X-Linked agammaglobulinemia of buonThere is defective humoral immunity with normal cell mediated immunity. It is characterized by the failure of B cell precursors (Pro-B cells and pre B cells) to mature into B cells. There is mutation in cytoplasmic tyrosine kinase called B - cell tyrosine kinase (BtK).The disease usually does not become apparent until about age 6 months, when maternal immunoglobulins are depleted.B cells are absent or markedly decreased in the circulation, and the serum levels of all classes of immunoglobulin.There is depletions of B cell area of lymphnode (Coical follicles and medullary cord) and spleen (perifollicular region, germinal centre and mantle layer). Tonsil and adenoids are atrophic.T-cell collection areas are normal i.e. (i) Paracoical area in lymph nodes, and (ii) Periaerial lymphoid region & malpighian corpuscle in white pulp of spleen.The disease is seen almost entirely in males. There is recurrent bacterial infections of respiratory tract by H. influenzae, str. pneumoniae or staph. aureus. There is increased susceptibity to infection with enterovirus (echo, coxsackievirus, poliovirus), giardia lamblia, and ahritis by mycoplasma.As there is agammaglobulinemia, Opsonization is defective (Immunoglobulins are the major opsonins in body).
B-cell defect
IgA deficiency
Ig M deficiency
Ig G deficiency313