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2015 ; 5
(3
): 204-12
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Macrophage Infiltration into the Glomeruli in Lipoprotein Glomerulopathy
#MMPMID26955632
Takasaki S
; Maeda K
; Joh K
; Yamakage S
; Fukase S
; Takahashi T
; Suzuki M
; Matsunaga A
; Saito T
Case Rep Nephrol Dial
2015[Sep]; 5
(3
): 204-12
PMID26955632
show ga
Lipoprotein glomerulopathy (LPG) is characterized by histopathological features
showing intra-glomerular lipoprotein thrombi and type III hyperlipoproteinemia
(HLP), with heterozygote mutation of apolipoprotein (apo) E gene. On the other
hand, as another renal lipidosis with type III HLP, apoE2 homozygote-related
glomerulopathy (apoE2-GN) showing foamy macrophages has been reported. The case
of a 25-year-old man who had LPG by clinical behavior and gene analysis, but
demonstrated atypical histopathological features with a substantial amount of
foamy macrophage infiltration in the glomeruli, is presented. The combination of
alleles for apoE Tokyo/Maebashi and classical apoE2 (Arg158Cys) was inferred to
be the leading cause of the unique renal pathology with lipoprotein thrombi and
foamy macrophages. In addition, foamy macrophages infiltrated some part of the
apoE-positive region within the glomerulus, but did not exist in lipoprotein
thrombi despite apoE positivity, suggesting that properties of apoE are crucial
in the development of LPG rather than macrophage function. This case provides
important information related to the pathogenesis of LPG and apoE2-GN.