A group of disorders characterized by imbalances in gamma globulins. The hallmark is that certain Ig levels in the patient's blood are extremely low or absent, while another Ig may be elevated; or Ig levels may be normal but functionally abnormal, among other conditions. The specific Ig deficiencies in patients can also change over time. The common immune defect is the inability of B cells to synthesize certain Igs or the production of only dysfunctional Igs. The number of B cells in the patient's peripheral blood may be reduced or normal, but there are intrinsic defects in B cell maturation and differentiation or abnormalities in T cell immune regulation.

bubble_chart Clinical Manifestations

The disease onset is relatively late, mostly seen in children and young adults, with no gender difference. Patients have a slightly lower susceptibility to infections compared to congenital agammaglobulinemia. The infection process is often chronic. The most common manifestation is recurrent purulent respiratory infections, frequently including chronic sinusitis and chronic pneumonia. Some patients (those with IgA deficiency) may present with malabsorption syndrome and often have intestinal giardiasis. Additionally, patients may be accompanied by autoimmune thyroiditis, granulocytopenia, pernicious anemia, rheumatoid arthritis, etc. The size of the tonsils and lymph nodes may be normal, and lymphoid follicles may be present in the lymph node cortex, with the spleen often enlarged.

bubble_chart Auxiliary Examination

1. Abnormal levels of serum immunoglobulins IgG, IgA, and IgM.

2. B cell counts are usually normal.

3. Some patients are accompanied by decreased cellular immune function.

It is basically the same as congenital agammaglobulinemia. The main treatments are gamma globulin replacement therapy (or fresh plasma transfusion) and antibacterial drugs. For those with intestinal malabsorption syndrome, dietary therapeutics are provided.