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A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension

BACKGROUND: Lipoprotein glomerulopathy (LPG) is a rare inherited renal disease characterized by intraglomerular lipoprotein within the lumina of severely dilated glomerular capillaries. The common clinical presentation of LPG includes proteinuria or nephrotic syndrome. Hypertension and anemia were t...

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Autores principales: Wu, Yu, Chen, Xiaohan, Yang, Yuan, Wang, Baohe, Liu, Xiaoxia, Tao, Ye, Fu, Ping, Hu, Zhangxue
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3598816/
https://www.ncbi.nlm.nih.gov/pubmed/23448537
http://dx.doi.org/10.1186/1471-2369-14-53
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author Wu, Yu
Chen, Xiaohan
Yang, Yuan
Wang, Baohe
Liu, Xiaoxia
Tao, Ye
Fu, Ping
Hu, Zhangxue
author_facet Wu, Yu
Chen, Xiaohan
Yang, Yuan
Wang, Baohe
Liu, Xiaoxia
Tao, Ye
Fu, Ping
Hu, Zhangxue
author_sort Wu, Yu
collection PubMed
description BACKGROUND: Lipoprotein glomerulopathy (LPG) is a rare inherited renal disease characterized by intraglomerular lipoprotein within the lumina of severely dilated glomerular capillaries. The common clinical presentation of LPG includes proteinuria or nephrotic syndrome. Hypertension and anemia were thought to be mild in LPG. Thrombotic microangiopathy (TMA) in LPG has not been previously reported. In this report, we present a patient with LPG that developed TMA. To the best of our knowledge, this is the first report of TMA in LPG. CASE PRESENTATION: Four years ago (2005), a 19-year-old Chinese woman was diagnosed with nephrotic syndrome and provided prednisone treatment. A combination of prednisone and cyclophosphamide did not have any effect and was discontinued after six months. Although she was steroid-resistant, over the next subsequent three years, she maintained normal renal function without anemia and thrombocytopenia. In February 2009, she had a severe headache and blurry vision and presented at a local hospital with severe hypertension. Blood pressure was 220/160 mmHg. Laboratory data showed hemoglobin 3.8 g/dL; platelet counts 29×10(9)/L; urinary protein 7.90 g/d; total bilirubin 29.9 umol/L; indirect bilirubin 28.2 umol/L; LDH 1172 U/L; ALB 2.66 g/dL; urea nitrogen 52 mg/dL; serum creatinine 3.2 mg/dL; triglyceride 253 mg/dL; total cholesterol 273 mg/dL. ANA, ds-DNA, ANCA, anti-GBM antibody and anticardiolipin were all negative. A renal biopsy revealed LPG with TMA. Genetic evaluation showed the patient carried the APOE Kyoto mutation. Adequate control of blood pressure improved microangiopathic anemia and thrombocytopenia, however, renal function did not improve and she eventually developed uremia and became hemodialysis dependent. CONCLUSION: We report on a rare case of TMA probably due to malignant hypertension in LPG. Early lipid-lowering and antihypertensive treatment may improve outcome. The pathophysiologic relationship between LPG and TMA should be investigated further.
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spelling pubmed-35988162013-03-16 A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension Wu, Yu Chen, Xiaohan Yang, Yuan Wang, Baohe Liu, Xiaoxia Tao, Ye Fu, Ping Hu, Zhangxue BMC Nephrol Case Report BACKGROUND: Lipoprotein glomerulopathy (LPG) is a rare inherited renal disease characterized by intraglomerular lipoprotein within the lumina of severely dilated glomerular capillaries. The common clinical presentation of LPG includes proteinuria or nephrotic syndrome. Hypertension and anemia were thought to be mild in LPG. Thrombotic microangiopathy (TMA) in LPG has not been previously reported. In this report, we present a patient with LPG that developed TMA. To the best of our knowledge, this is the first report of TMA in LPG. CASE PRESENTATION: Four years ago (2005), a 19-year-old Chinese woman was diagnosed with nephrotic syndrome and provided prednisone treatment. A combination of prednisone and cyclophosphamide did not have any effect and was discontinued after six months. Although she was steroid-resistant, over the next subsequent three years, she maintained normal renal function without anemia and thrombocytopenia. In February 2009, she had a severe headache and blurry vision and presented at a local hospital with severe hypertension. Blood pressure was 220/160 mmHg. Laboratory data showed hemoglobin 3.8 g/dL; platelet counts 29×10(9)/L; urinary protein 7.90 g/d; total bilirubin 29.9 umol/L; indirect bilirubin 28.2 umol/L; LDH 1172 U/L; ALB 2.66 g/dL; urea nitrogen 52 mg/dL; serum creatinine 3.2 mg/dL; triglyceride 253 mg/dL; total cholesterol 273 mg/dL. ANA, ds-DNA, ANCA, anti-GBM antibody and anticardiolipin were all negative. A renal biopsy revealed LPG with TMA. Genetic evaluation showed the patient carried the APOE Kyoto mutation. Adequate control of blood pressure improved microangiopathic anemia and thrombocytopenia, however, renal function did not improve and she eventually developed uremia and became hemodialysis dependent. CONCLUSION: We report on a rare case of TMA probably due to malignant hypertension in LPG. Early lipid-lowering and antihypertensive treatment may improve outcome. The pathophysiologic relationship between LPG and TMA should be investigated further. BioMed Central 2013-02-28 /pmc/articles/PMC3598816/ /pubmed/23448537 http://dx.doi.org/10.1186/1471-2369-14-53 Text en Copyright ©2013 Wu et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Wu, Yu
Chen, Xiaohan
Yang, Yuan
Wang, Baohe
Liu, Xiaoxia
Tao, Ye
Fu, Ping
Hu, Zhangxue
A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension
title A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension
title_full A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension
title_fullStr A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension
title_full_unstemmed A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension
title_short A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension
title_sort case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3598816/
https://www.ncbi.nlm.nih.gov/pubmed/23448537
http://dx.doi.org/10.1186/1471-2369-14-53
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