Overview.
Late-onset hypogammaglobulinemia is a group of gammaglobulin imbalances characterized by patients who have extremely low or deficient levels of certain immunoglobulins (Ig) in their blood, while another Ig may be elevated. The patient may also have normal levels of Igs that do not function properly. The patient’s Ig defects may also change over time, and the common immunodeficiency is the inability of B cells to synthesize certain Igs or to synthesize only dysfunctional Igs.The patient may have a reduced or normal number of B cells in the peripheral blood, but there is an intrinsic defect in the maturation and differentiation of the B cells, or there may be a malfunction in the immunoregulation of the T cells.The patient may have an Ig deficiency in the peripheral blood.
Etiology
The common immunodeficiency is the inability of B cells to synthesize certain Igs or to synthesize only dysfunctional Igs. patients have a reduced or normal number of B cells in the peripheral blood, but there is an intrinsic defect in B cell maturation and differentiation or a malfunctioning of T cell immunoregulation.
Symptoms
Delayed-onset hypoprocythemia has a slow onset and is most common in children and young adults, with no gender differences. Patients are slightly less susceptible to infections than in congenital gammaglobulin deficiency and tend to have a chronic course of infections. The most common ones are recurrent purulent respiratory tract infections, mostly chronic paranasal sinusitis and chronic pneumonia. Some patients (those with IgA deficiency) are associated with malabsorption syndrome and often have intestinal Giardia lamblia. In addition, patients may have autoimmune thyroiditis, granulocytopenia, pernicious anemia, and rheumatoid arthritis. Tonsils and lymph nodes may be normal in size, lymphoid follicles may be present in the lymph node cortex, and the spleen is often enlarged.
Examination
1. Abnormal serum immunoglobulin IgG, IgA, IgM levels.
2. B-cell values are often normal.
3. Some patients have reduced cellular immunity.
Diagnosis
Diagnosis can be made on the basis of history, clinical manifestations and laboratory data.
Treatment
The treatment is basically the same as that for congenital gammaglobulin deficiency. The main treatment is to apply gammaglobulin replacement therapy (or transfusion of fresh plasma) and antibacterial drugs. Dietary therapy is given to those with intestinal malabsorption syndrome.