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LM Endo,1,2 JV
Giannobile,1,2 AK Dobbs,3 JB
Foote,4 E Szymanska,4 DG Warnock,2
WJ Cook,5 ME Conley,3 HW Schroeder2,4,6 |
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1Department of Pediatrics, University
of Alabama at Birmingham, Birmingham, Alabama, USA
2Department of Medicine, University of Alabama at
Birmingham, Birmingham, Alabama, USA
3Immunology Department, St. Jude Childrens Research
Hospital, Memphis, Tennessee, USA
4Department of Microbiology, University of Alabama at
Birmingham, Birmingham, Alabama, USA
5Department of Pathology, University of Alabama at
Birmingham, Birmingham, Alabama, USA
6Department of Genetics, University of Alabama at
Birmingham, Birmingham, Alabama, USA |
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Abstract |
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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
reflect IVIG
reacting with
polymorphic
autoantigens, an
endogenous IgG-producing
clone reacting with
a common idiotype
present in the IVIG,
or both.
Key words:
X-linked
agammaglobulinemia (XLA).
Bruton
agammaglobulinemia.
Membranous
glomerulopathy.
Microhematuria.
Intravenous
gammaglobulin.
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