Retroperitoneal fibrosis in three siblings with the sickle cell trait

Can Med Assoc J. 1973 Apr 21;108(8):1025-9.


Three West-Indian black siblings with the sickle cell trait developed retroperitoneal fibrosis, a previously unreported association. Other well known renal manifestations associated with the sickle cell trait were also present in some of these cases and included renal medullary necrosis and spontaneous hematuria. It is postulated that the sickling of the erythrocytes in the periureteral vessels resulted in thrombosis, ischemia, reactive scarring and progressive fibrosis indistinguishable from the known histological picture of retroperitoneal fibrosis. The finding of fibrin thrombi in the small veins of the fibrotic tissue of one of these patients would support this explanation.

MeSH terms

  • Adult
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / genetics
  • Anemia, Sickle Cell / immunology
  • Anemia, Sickle Cell / pathology
  • Biopsy
  • Female
  • Hematuria / etiology
  • Hemoglobins, Abnormal / analysis
  • Humans
  • Immunoglobulins / analysis
  • Kidney Papillary Necrosis / etiology
  • Male
  • Retroperitoneal Fibrosis / etiology*
  • Retroperitoneal Fibrosis / genetics
  • Retroperitoneal Fibrosis / immunology
  • Retroperitoneal Fibrosis / pathology


  • Hemoglobins, Abnormal
  • Immunoglobulins