Acute renal failure associated to paroxysmal nocturnal haemoglobinuria leads to intratubular haemosiderin accumulation and CD163 expression

Nephrol Dial Transplant. 2011 Oct;26(10):3408-11. doi: 10.1093/ndt/gfr391. Epub 2011 Jul 19.

Abstract

Decreased renal function has been observed in diseases with intravascular haemolysis, including paroxysmal nocturnal haemoglobinuria (PNH). However, the mechanisms via which haemoglobin enhances renal damage in this pathology are not fully known. We report a case of acute renal failure associated to PNH and extensive haemosiderin deposits in tubular cells. Renal biopsy also revealed a strong immunostaining of CD163 (a haemoglobin scavenger receptor expressed in macrophages) and oxidative stress markers (NADPH-p22 phox and haeme oxigenase-1) in areas with deposits of iron. This fact provides evidence for a pathogenic role for free haemoglobin in tubulointerstitial renal injury in human PNH disease.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Kidney Injury / complications*
  • Adult
  • Antigens, CD / metabolism
  • Antigens, Differentiation, Myelomonocytic / metabolism
  • Biomarkers / metabolism*
  • Heme Oxygenase-1 / metabolism
  • Hemoglobins / metabolism
  • Hemoglobinuria, Paroxysmal / diagnosis*
  • Hemoglobinuria, Paroxysmal / etiology*
  • Hemosiderin / metabolism*
  • Humans
  • Immunoenzyme Techniques
  • Iron / metabolism
  • Kidney Tubules / physiopathology*
  • Macrophages / metabolism
  • Male
  • NADP / metabolism
  • NADPH Oxidases / metabolism
  • Receptors, Cell Surface / metabolism
  • Young Adult

Substances

  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • Biomarkers
  • CD163 antigen
  • Hemoglobins
  • Receptors, Cell Surface
  • NADP
  • Hemosiderin
  • Iron
  • Heme Oxygenase-1
  • NADPH Oxidases
  • CYBA protein, human