- Published on
Pathology - IgA Deficiency
IgA deficiency is a frequent genetic condition causing varying hypogammaglobulinemia. Patients may acquire a hypogammaglobulinemia at any age and may be asymptomatic. The condition develops when the respective immunoglobulin-producing B cells fail to mature into plasma cells. Patients have normal B cell numbers, but measurably reduced amounts of one particular kind of immunoglobulin. This instance depicts the classic symptomatic patient. Deficiency in secretory IgA (immunoglobulin A) at very low levels results in recurrent sinopulmonary and, most notably, Giardia infections. This patient is considerably dehydrated on physical examination, which can occur following considerable fluid loss from giardiasis. It is also important to recognize that anaphylaxis can occur if an IgAdeficient patient is exposed to blood products that contain IgA, which may be the first recognition of previously asymptomatic IgA-deficient adult. In addition, patients have elevated rates of allergy and autoimmune illnesses.
A similar presentation in an adult patient with decreased levels of numerous immunoglobulins (as opposed to a single immunoglobulin deficiency in IgA deficiency) is mixed variable immunodeficiency, from a failure in B-cell maturation to plasma cells.
IgA deficiency is a frequent genetic condition causing varying hypogammaglobulinemia. Patients may acquire a hypogammaglobulinemia at any age and may be asymptomatic. The condition develops when the respective immunoglobulin-producing B cells fail to mature into plasma cells. Patients have normal B cell numbers, but measurably reduced amounts of one particular kind of immunoglobulin. This instance depicts the classic symptomatic patient. Deficiency in secretory IgA (immunoglobulin A) at very low levels results in recurrent sinopulmonary and, most notably, Giardia infections. This patient is considerably dehydrated on physical examination, which can occur following considerable fluid loss from giardiasis. It is also important to recognize that anaphylaxis can occur if an IgAdeficient patient is exposed to blood products that contain IgA, which may be the first recognition of previously asymptomatic IgA-deficient adult. In addition, patients have elevated rates of allergy and autoimmune illnesses.
A similar presentation in an adult patient with decreased levels of numerous immunoglobulins (as opposed to a single immunoglobulin deficiency in IgA deficiency) is mixed variable immunodeficiency, from a failure in B-cell maturation to plasma cells.
0 Comments
