title: Membranous glomerulopathy in an adult patient with X-linked agammaglobulinemia receiving intravenous gammaglobulin.
abstract: BACKGROUND
  Immune complex deposition in the subepithelial zone of glomerular capillaries can lead to membranous glomerulopathy.
  
  
  OBJECTIVE
  To present the case of a 23-year-old man with X-linked agammaglobulinemia (XLA) who developed idiopathic membranous glomerulopathy while receiving intravenous immunoglobulin (IVIG).
  
  
  METHODS
  We performed an immunological workup, genetic testing, and a renal biopsy.
  
  
  RESULTS
  XLA was confirmed with less than 0.02% CD19+ cells in the blood after sequence analysis revealed a nonfunctional BTK gene. The patient presented with microhematuria, which persisted for 3 years and spanned treatment with 5 different preparations of intravenous gammaglobulin. Immunohistochemistry revealed membranous glomerulopathy.
  
  
  CONCLUSIONS
  Although endogenous serum immunoglobulin (Ig) production is severely impaired in XLA, rare B lymphocytes that have managed to mature can produce functional IgG antibodies. The pathogenic immune complexes could reflect IVIG reacting with polymorphic autoantigens, an endogenous IgG-producing clone reacting with a common idiotype present in the IVIG, or both.
authors: Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama 35294-2182, USA.
authors: L M
authors: LM
authors: Endo
authors: J V
authors: JV
authors: Giannobile
authors: A K
authors: AK
authors: Dobbs
authors: J B
authors: JB
authors: Foote
authors: E
authors: E
authors: Szymanska
authors: D G
authors: DG
authors: Warnock
authors: W J
authors: WJ
authors: Cook
authors: M E
authors: ME
authors: Conley
authors: H W
authors: HW
authors: Schroeder
mesh_terms: D000077329:Agammaglobulinaemia Tyrosine Kinase; D000361:Agammaglobulinemia / Q000150:complications* / Q000235:genetics / Q000276:immunology / Q000628:therapy; D000888:Antibodies, Anti-Idiotypic / Q000378:metabolism; D001706:Biopsy; D004252:DNA Mutational Analysis; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000235:genetics / Q000276:immunology / Q000628:therapy; D015433:Glomerulonephritis, Membranous / Q000209:etiology* / Q000235:genetics / Q000276:immunology / Q000628:therapy; D006801:Humans; D056724:Immunity, Humoral / Q000235:genetics; D016756:Immunoglobulins, Intravenous / Q000009:adverse effects* / Q000627:therapeutic use; D007668:Kidney / Q000276:immunology / Q000378:metabolism* / Q000473:pathology; D008297:Male; D011505:Protein-Tyrosine Kinases / Q000235:genetics*; D055815:Young Adult
chemical_list: D000888:Antibodies, Anti-Idiotypic; D016756:Immunoglobulins, Intravenous; D011505:Protein-Tyrosine Kinases; D000077329:Agammaglobulinaemia Tyrosine Kinase; C000625949:BTK protein, human