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Membranous glomerulopathy in an adult patient with X-linked agammaglobulinemia receiving intravenous gammaglobulin
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نویسنده
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endo l.m. ,giannobile j.v. ,dobbs a.k. ,foote j.b. ,szymanska e. ,warnock d.g. ,cook w.j. ,conley m.e. ,schroeder h.w.
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منبع
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journal of investigational allergology and clinical immunology - 2011 - دوره : 21 - شماره : 5 - صفحه:405 -409
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چکیده
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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. conclusion: 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 refl ect ivig reacting with polymorphic autoantigens,an endogenous igg-producing clone reacting with a common idiotype present in the ivig,or both.
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کلیدواژه
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Bruton agammaglobulinemia; Intravenous gammaglobulin; Membranous glomerulopathy; Microhematuria; X-linked agammaglobulinemia (XLA)
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آدرس
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department of pediatrics,university of alabama at birmingham,birmingham,al,united states,department of medicine,university of alabama at birmingham,birmingham,al, United States, department of pediatrics,university of alabama at birmingham,birmingham,al,united states,department of medicine,university of alabama at birmingham,birmingham,al, United States, immunology department,st. jude children's research hospital,memphis,tn, United States, department of microbiology,university of alabama at birmingham,birmingham,al, United States, department of microbiology,university of alabama at birmingham,birmingham,al, United States, department of medicine,university of alabama at birmingham,birmingham,al, United States, department of pathology,university of alabama at birmingham,birmingham,al, United States, immunology department,st. jude children's research hospital,memphis,tn, United States, department of medicine,university of alabama at birmingham,birmingham,al,united states,department of microbiology,university of alabama at birmingham,birmingham,al,united states,department of genetics,university of alabama at birmingham,birmingham,al, United States
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Authors
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