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

Yu Wu1, Xiaohan Chen, Yuan Yang

  • 1Department of Hematology, West China Hospital, National Key Laboratory of Biotherapy of Human Diseases, Sichuan University, Chengdu, Sichuan Province, China.

BMC Nephrology
|March 2, 2013
PubMed

Insights

Lipoprotein glomerulopathy (LPG) is a rare kidney disease. This case report details the first instance of thrombotic microangiopathy (TMA) in LPG, likely triggered by severe hypertension, highlighting the need for further research.

Area of Science:

  • Nephrology
  • Genetics
  • Vascular Biology

Background:

  • Lipoprotein glomerulopathy (LPG) is a rare inherited renal disease characterized by intraglomerular lipoprotein accumulation.
  • Typical LPG presentation includes proteinuria or nephrotic syndrome, with usually mild hypertension and anemia.
  • Thrombotic microangiopathy (TMA) has not been previously reported in LPG patients.

Observation:

  • A 19-year-old woman with steroid-resistant nephrotic syndrome and LPG developed severe hypertension (220/160 mmHg), anemia (Hb 3.8 g/dL), and thrombocytopenia (29 × 10(9)/L).
  • Renal biopsy confirmed LPG with TMA.
  • Genetic testing revealed the APOE Kyoto mutation.

Findings:

  • The patient presented with malignant hypertension, microangiopathic anemia, and thrombocytopenia, associated with LPG.
  • Despite blood pressure control improving TMA symptoms, renal function declined, leading to end-stage renal disease requiring hemodialysis.
  • The APOE Kyoto mutation was identified in the patient.

Implications:

  • This is the first reported case of thrombotic microangiopathy (TMA) in lipoprotein glomerulopathy (LPG).
  • Malignant hypertension may be a trigger for TMA in LPG patients.
  • Early lipid-lowering and antihypertensive therapies may improve outcomes in LPG patients with TMA; further investigation into the LPG-TMA pathophysiologic link is warranted.
Abstract

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