Evaluating and managing hypogammaglobulinemia.

Abstract

If a patient has frequent or recurrent bronchopulmonary or sinus infections, they may be due to low levels of immunoglobulins. This article describes common primary (idiopathic) and secondary forms of hypogammaglobulinemia and how to evaluate and manage them.

Should mild hypogammaglobulinemia be managed as severe hypogammaglobulinemia? A study of 389 patients with secondary hypogammaglobulinemia

“…Common causes of hypogammaglobulinemia such as hematological diseases (MM, CLL, B-cell lymphoma, AL amyloidosis, monoclonal gammopathy of unknown significance (MGUS)), drugs, Ig leakages (nephrotic syndrome, protein-losing enteropathy, capillary leakage), and infections (HIV, EBV, CMV, parvovirus B19) [1,2,13] were identified. Demographic data, biological findings, vaccination against S. pneumoniae and intravenous Ig (IVIg) therapy were recorded.…”

“…Hypogammaglobulinemia can be due to a variety of conditions, which can be divided into immunoglobulin production diseases such as lymphoid hemopathies (most commonly chronic lymphocytic leukemia (CLL) and multiple myeloma (MM)), infections (human immunodeficiency virus (HIV), parvovirus B19, Epstein-Barr virus (EBV), cytomegalovirus (CMV)) [1,2], medications (mainly antiepileptic and immunosuppressive therapies (IST)) and immunoglobulin loss diseases such as enteropathies or renal amyloidosis. When no cause is identified, a diagnosis of primary immune deficiency, most frequently common variable immunodeficiency (CVID) or Good syndrome in adults, has to be made [1,2].…”

Guía colombiana de práctica clínica para el uso de inmonuglobulinas en el tratamiento de reemplazo e inmunomodulación

“…(GRADE A1) 65,70 Enfermedades con pérdida de proteínas R Se ha documentado hipogammaglobulinemia e infecciones secundarias asociadas con diversas patologías, que incrementan la pérdida de proteínas a nivel intestinal (enteropatía perdedora de proteínas, linfangiectasias), renal (síndrome nefrótico, nefritis asociada a enfermedades autoinmunes), torácico (quilotórax) y piel (quemaduras); sin embargo, no existen estudios controlados que evalúen la utilidad de la IgIV o la inmunoglobulina subcutánea en estos pacientes. (GRADE C2) 71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87 Inmunodeficiencias secundarias al uso de anticonvulsivantes R Se ha observado hipogammaglobulinemia en individuos que reciben tratamiento con anticonvulsivantes (carbamacepina, fenitoína, lamotrigina, entre otros). El tratamiento de reemplazo está indicado en pacientes con niveles bajos de IgG y con infecciones graves o recurrentes cuando no pueden emplearse otros anticonvulsivantes o cuando la hipogammaglobulinemia persiste después de suspenderlos.…”

Value of reflex testing based on hypogammaglobulinemia as demonstrated in serum protein electrophoresis

“…Causes of hypo-Ig are varied and maybe primary (genetic or idiopathic) or secondary to a large number of disease conditions. The latter encompass conditions that may be associated with (1) increased Ig loss or catabolism such as nephrotic syndrome and other severe renal diseases, severe burns, sepsis, and protein-losing enteropathy (which includes autoimmune enteropathy and intestinal lymphangiectasia), (2) reduced Ig production due to malignancies (e.g., chronic lymphocytic leukemia, lymphoma, multiple myeloma), malnutrition, rheumatoid diseases (rheumatoid arthritis, systemic lupus erythematosus), viral infections (HIV, Epstein-Barr virus, rubella, cytomegalovirus), (3) medications (antiepileptic, immunosuppressive, chemotherapeutic, antirheumatic, systemic steroids, etc. ), and (4) high-stress [1][2][3].…”

“…The latter encompass conditions that may be associated with (1) increased Ig loss or catabolism such as nephrotic syndrome and other severe renal diseases, severe burns, sepsis, and protein-losing enteropathy (which includes autoimmune enteropathy and intestinal lymphangiectasia), (2) reduced Ig production due to malignancies (e.g., chronic lymphocytic leukemia, lymphoma, multiple myeloma), malnutrition, rheumatoid diseases (rheumatoid arthritis, systemic lupus erythematosus), viral infections (HIV, Epstein-Barr virus, rubella, cytomegalovirus), (3) medications (antiepileptic, immunosuppressive, chemotherapeutic, antirheumatic, systemic steroids, etc. ), and (4) high-stress [1][2][3]. Individuals with consistently reduced serum Ig concentrations are more susceptible to infections [4][5][6][7][8], and may be associated with an increased risk of developing cancer, particularly hematological neoplasms, as observed in patients with common variable immunodeficiency [9][10][11].…”