Original Article
Hemolytic uremic syndrome complicated with IgA nephropathy: a clinicopathological study
Yao Xingfeng, Wang Xiang, Fu Libing, Chen Zhi, Zhang Nan, Li Xueqian, Liu Xiaorong, He Lejian
Published 2021-08-08
Cite as Chin J Pathol, 2021, 50(8): 910-914. DOI: 10.3760/cma.j.cn112151-20210118-00048
Abstract
ObjectiveTo investigate the clinicopathologic characteristics, treatments, outcomes and mechanisms of hemolytic uremic syndrome (HUS) complicated with IgA nephropathy (IgAN).
MethodsThe clinical manifestations, treatments, prognosis and histopathological features of renal biopsy tissues were analyzed in two cases of HUS complicated with IgAN from Beijing Children′s Hospital, Capital Medical University using light microscopy, immunofluorescence detection and electron microscopy. The related literatures were also reviewed.
ResultsThe clinical manifestations were microvascular hemolytic anemia, thrombocytopenia, acute renal impairment with hematuria, proteinuria, and positive anti-H factor antibody. Histological findings confirmed presence of both HUS and IgAN. Histological features included glomerular mesangial and stromal hyperplasia with endothelial cell proliferation, capillary stenosis, arteriolar thickening, and glomerular ischemia and capillary dilatation. Immunofluorescence detection showed diffuse IgA deposition in the glomerular mesangial matrix. Electron microscopy showed proliferation of mesangial and endothelial cells, thickening of the inner layer of the glomerular basement membrane, deposition of massive electronic densification in the mesangial region, and shrinkage of the segmental basement membrane. The two children were very responsive to plasma exchange and steroid treatments. However, their urine protein and occult blood tests remained continuously positive during the follow-up of 5 years 7 months and 8 months respectively.
ConclusionsHUS complicated with IgAN is rare. The diagnosis relies on various pathological examinations, which require the combination of light microscopy, immunofluorescence detection and electron microscopy. Plasma exchange and steroid treatments are effective. However, the long-term prognosis is concerning and may relate to pathological grade and secondary factors. The mechanism of connecting HUS and IgAN is unknown, but may be caused by prodromal or secondary factors.
Key words:
Hemolytic-Uremic Syndrome; Glomerulonephritis, IGA; Electron microscope
Contributor Information
Yao Xingfeng
Department of Pathology,Beijing Children′s Hospital,Capital Medical University, National Center for Children′s Health
Beijing 100045
Wang Xiang
Department of Pathology,Beijing Children′s Hospital,Capital Medical University, National Center for Children′s Health
Beijing 100045
Fu Libing
Department of Pathology,Beijing Children′s Hospital,Capital Medical University, National Center for Children′s Health
Beijing 100045
Chen Zhi
Department of Reanal Medicine,Beijing Children′s Hospital,Capital Medical University, National Center for Children′s Health
Beijing 100045, China
Zhang Nan
Department of Pathology,Beijing Children′s Hospital,Capital Medical University, National Center for Children′s Health
Beijing 100045
Li Xueqian
Department of Reanal Medicine,Beijing Children′s Hospital,Capital Medical University, National Center for Children′s Health
Beijing 100045, China
Liu Xiaorong
Department of Reanal Medicine,Beijing Children′s Hospital,Capital Medical University, National Center for Children′s Health
Beijing 100045, China
He Lejian
Department of Pathology,Beijing Children′s Hospital,Capital Medical University, National Center for Children′s Health
Beijing 100045
