Definition
- Hypogammaglobulinemia: congenital or acquired decrease or absence in IgG classes
Etiology
- Ataxia-Telangiectasia
- Autosomal Recessive Agammaglobulinemia (ARA)
- B-Cell Lineage Malignancies: increased catabolism of immunoglobulins
- Biallelic Deficiency of Mismatch Repair Protein PMS2
- Bruton Agammaglobulinemia (X-Linked Aggamglobulinemia) (see Bruton Agammaglobulinemia, [[Bruton Agammaglobulinemia]]): most cases do not present prior to 6 mo of age
- Common Variable Immunodeficiency (CVID) (see Common Variable Immunodeficiency, [[Common Variable Immunodeficiency]]): most cases do not present until the third or fourth decade of life
- Drug-Related Hypogammaglobulinemia: these cases probably occur in specific predisposed individuals
- Captopril (see Captopril, [[Captopril]]): may cause IgA deficiency
- Carbamazepine (Tegretol) (see Carbamazepine, [[Carbamazepine]]): may cause IgA deficiency or reversible hypogammaglobulinemia
- Chlorpromazine (Thorazine) (see Chlorpromazine, [[Chlorpromazine]]): may cause IgA deficiency
- Hydroxychloroquine (Plaquenil) (see Hydroxychloroquine, [[Hydroxychloroquine]]): may cause IgA deficiency
- Lamotrigine (Lamictal) (see Lamotrigine, [[Lamotrigine]]): may cause reversible hypogammaglobulinemia
- Penicillamine (see Penicillamine, [[Penicillamine]]): may cause IgA deficiency
- Phenytoin (Dilantin) (see Phenytoin, [[Phenytoin]]): may cause IgA deficiency or reversible hypogammaglobulinemia
- Sulfasalazine (see Sulfasalazine, [[Sulfasalazine]]): may cause IgA deficiency
- Valproic Acid (Depakote, Depakene) (see Valproic Acid, [[Valproic Acid]]): may cause IgA deficiency
- Good Syndrome (Immunodeficiency with Thymoma) (see Thymoma, [[Thymoma]])
- Hyper-IgM Syndrome
- CD40 Ligand (CD40L or CD154) Deficiency: this X-linked disorder is the etiology of most cases of Hyper-IgM syndrome
- CD40 Deficiency
- Activation-Induced Cytidine Deaminase (AID) Deficiency
- Uracil-Nucleoside-Glycosylase (UNG) Deficiency
- Immunodeficiency–Centromeric Instability–Facial Anomalies (ICF) Syndrome: normal B-cell counts with agammaglobulinemia
- Jacobsen Syndrome (Hemizygous Deletion of Part of the Long Arm of Chromosome 11)
- Immunosuppression
- Autologous Stem Cell Transplant (SCT) (see Bone Marrow Transplant, [[Bone Marrow Transplant]])
- Chemotherapy
- Corticosteroids (see Corticosteroids, [[Corticosteroids]])
- Patients taking ≥12.5 mg prednisone for at least 1 year are at increased risk of hypogammaglobulinemia
- Patients with hypogammaglobulinemia due to corticosteroid usually retain specific antibody responses: therefore, they are not usually candidates for immunoglobulin replacement therapy
- Rituximab (see Anti-CD20 Therapy, [[Anti-CD20 Therapy]])
- Isolated IgA Deficiency (see Isolated IgA Deficiency, [[Isolated IgA Deficiency]]): few reported cases of bronchiectasis (although these were probably due to undetected IgG deficiencies): most common primary immunodeficiency syndrome (occurs in 1:600 persons)
- Lymphoproliferative Malignancy
- Chronic Lymphocytic Leukemia (CLL) (see Chronic Lymphocytic Leukemia, [[Chronic Lymphocytic Leukemia]]): commonly associated with hypogammaglobulinemia and infection
- Multiple Myeloma (see Multiple Myeloma, [[Multiple Myeloma]]): antibody deficiency with normal total IgG levels (due to contribution of the paraprotein to the total IgG level and due to tumor cells altering normal regulatory T cells, impairing B-cell maturation)
- Myelodysplastic Syndrome (see Myelodysplastic Syndrome, [[Myelodysplastic Syndrome]])
- Myotonic Dystrophy: increased catabolism of immunoglobulins
- Nephrotic Syndrome (see Nephrotic Syndrome, [[Nephrotic Syndrome]])
- Hypoalbuminemia (see Hypoalbuminemia, [[Hypoalbuminemia]])
- Edema: usually
- Prematurity in Infants: premature infants delivered before the third trimester usually lack adequate maternal immunoglobulin and may also more rapid metabolize the IgG that they have received
- Protein-Losing Enteropathy: intestinal lymphangiectasia, etc
- IgG levels are typically affected more than IgM or IgA levels (however, the levels of IgG, IgM, and IgA may all be decreased in severe protein-losing enteropathy)
- Hypoalbuminemia (see Hypoalbuminemia, [[Hypoalbuminemia]])
- Edema: usually
- Selective IgG Subclass Deficiency (see Selective IgG Subclass Deficiency, [[Selective IgG Subclass Deficiency]]): decrease in one or more of the four classes of IgG with normal total IgG is most common type associated with bronchiectasis
- Severe Burns: increased catabolism of immunoglobulins
- Severe Combined Immunodeficiency (SCID)
- Specific Antibody Deficiency (SAD)/Specific Polysaccharide Antibody Deficiency (SPAD) (see Specific Antibody Deficiency, [[Specific Antibody Deficiency]]): poor serological response to polysaccharide antigens (with normal levels of immunoglobulins and IgG subclasses) and normal responses to protein antigens
- Steinert’s Disease
- Transcobalamine Deficiency
- Trisomy 18
- Wiskott-Aldrich Syndrome
(radiation, malnutrition, and thyrotoxicosis are not beleived to be associated with hypogammaglobulinemia)
References
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