hypogammaglobulinemia; immunoglobulin deficiency

Etiology: 1) primary hypogammaglobulinemia - immunodeficiency syndromes 2) secondary hypogammaglobulinemia a) nephrotic syndrome b) protein-losing enteropathy Pathology: - may be markedly reduced, but not absent, B-cells Genetics: - associated with defects in CD19 - associated with defects in CD79B Clinical manifestations: - frequent infections - abdominal pain Laboratory: 1) quantitative immunoglobulins in serum 2) low serum albumin suggests secondary hypogammaglobulinemia 3) proteinuria suggests nephrotic syndrome Radiology: - chest X-ray & high-resolution computed tomography (CT) of thorax may show bronchiectasis Complications: - humoral immune dysfunction - bronchiectasis Management: -> immunoglobulin replacement -> not helpful in secondary hypogammaglobulinemia because proteins are rapidly lost in the urine or feces

Related

humoral immune dysfunction nephrotic syndrome

General

blood protein disorder immunodeficiency; immunodeficiency syndrome

References

Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998