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Haemoglobinuria is associated with chronic kidney disease and its progression in patients with sickle cell anaemia

Santosh L Saraf1, Xu Zhang, Tamir Kanias

  • 1Section of Hematology-Oncology, Department of Medicine, Comprehensive Sickle Cell Center, University of Illinois at Chicago, Chicago, IL, USA.

British Journal of Haematology
|December 17, 2013
PubMed

Insights

Haemoglobinuria, the presence of haemoglobin in urine, is common in sickle cell anaemia (SCA). This condition is linked to chronic kidney disease (CKD) progression and renal dysfunction in SCA patients.

Area of Science:

  • Nephrology
  • Hematology
  • Vascular Biology

Background:

  • Sickle cell anaemia (SCA) is a genetic blood disorder associated with various complications, including chronic kidney disease (CKD).
  • The role of haemoglobinuria, defined as haemoglobin in urine, in the pathogenesis of renal dysfunction in SCA is not fully understood.

Purpose of the Study:

  • To investigate the association between haemoglobinuria and the prevalence and progression of CKD in adult patients with SCA.
  • To explore the relationship between haemoglobinuria, lactate dehydrogenase, and CKD in SCA.

Main Methods:

  • Analysis of 356 adult SCA patients from the University of Illinois at Chicago (UIC) cohort and 439 from the Walk-PHaSST cohort.
  • CKD classification using National Kidney Foundation Kidney Disease Outcomes Quality Initiatives guidelines.
  • Haemoglobinuria detection via urine dipstick and confirmation by ELISA; pathway analysis to identify associations.

Main Results:

  • Prevalence of CKD was 58% (UIC) and 54% (Walk-PHaSST); haemoglobinuria was present in 36% (UIC) and 20% (Walk-PHaSST).
  • Lactate dehydrogenase showed an independent association with haemoglobinuria, which in turn was independently associated with CKD and age.
  • In the UIC cohort, haemoglobinuria significantly predicted CKD progression (HR 13.9) and increasing albuminuria (HR 3.1) over 32 months.

Conclusions:

  • Haemoglobinuria is a common finding in sickle cell anaemia.
  • Haemoglobinuria is significantly associated with both the presence and progression of chronic kidney disease in SCA patients.
  • Intravascular haemolysis, indicated by haemoglobinuria, likely plays a role in the development of renal dysfunction in SCA.

Keywords:
albuminuriahaemoglobinuriahaemolysiskidney diseasesickle cell

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