Decreased IL-1 beta and TNF alpha secretion in long-term bone marrow culture supernatant from Fanconi's anaemia patients

Eur J Haematol. 1996 Sep;57(3):202-7. doi: 10.1111/j.1600-0609.1996.tb01364.x.


Recent studies have shown that abnormalities of cytokine and lymphokine secretion are involved in the pathophysiology of Fanconi's anaemia (FA). In the present study, we quantified IL-1 beta, IL-1 receptor antagonist (IL-1Ra), IL-6 and TNF alpha protein levels in the supernatant of long-term cultures generated from BM cells of FA patients. Cell-free conditioned medium from long-term bone marrow culture was harvested every week at confluence and tested for interleukin secretion. IL-1 beta, IL-1Ra, TNF alpha and IL-6 protein secretion was assessed using immunoassays. IL-6 secretion was similar between controls and FA supernatants from wk 1 to wk 4. TNF alpha released from FA cells was consistently found at very low levels compared to control cells during the first 3 wk. Furthermore, secretion of IL-1 beta by cells from FA was always more than 2 standard deviations below the value of IL-1 beta found in normal donor cells from wk 1 to wk 4. In conclusion, in addition to a stem cell defect, a marked decrease in IL-1 beta and TNF alpha secretion may be one of the mechanisms leading to bone marrow failure in individuals with Fanconi's anaemia.

MeSH terms

  • Adolescent
  • Adult
  • Bone Marrow / pathology*
  • Cells, Cultured
  • Child
  • Fanconi Anemia / pathology*
  • Female
  • Humans
  • Interleukin-1 / metabolism*
  • Male
  • Receptors, Interleukin-1 / antagonists & inhibitors
  • Time Factors
  • Tumor Necrosis Factor-alpha / metabolism*


  • Interleukin-1
  • Receptors, Interleukin-1
  • Tumor Necrosis Factor-alpha