5q minus syndrome

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Classification according to ICD-10
D46 5q minus syndrome (myelodysplastic syndrome)
ICD-O 9986/3
Q93.5 Other deletions of a part of a chromosome
ICD-10 online (WHO version 2019)

The 5q-minus syndrome belongs to the group of myelodysplastic syndromes (MDS). It is characterized cytogenetically by the loss of part of chromosome 5 (long arm of chromosome 5 = 5q) and therefore got its name.

Bone marrow histology with the typical “non-lobulated” megakaryocytes in the 5q-minus syndrome

The clinical symptoms of the 5q-minus syndrome are essentially the same as those of the myelodysplastic syndromes. Anemia ( anemia ) and leukopenia (lack of white blood cells) develop , but the platelet counts are usually normal or even increased. This results in the clinical symptoms of weakness, exhaustion, susceptibility to infection and, if necessary, a tendency to bleeding. The bone marrow examination typically reveals changes in the megakaryocytes that are increased overall but have unusually small, non-lobed nuclei .

As with all MDS, there is a risk of developing acute myeloid leukemia , but the 5q-minus syndrome is considered a “low-risk MDS” with a comparatively favorable prognosis. There are promising treatment results with the new drug lenalidomide (Revlimid), a derivative of thalidomide , which give hope for a significant improvement in the prognosis. Lenalidomide was approved by the FDA for the treatment of 5q-minus syndrome in the United States in 2005 . However, the application for approval from the manufacturer of Lenalidomid Celgene was assessed negatively by the European Medicines Agency (EMA) in June 2008 and was therefore withdrawn by Celgene before the final decision. The EMA reviewers mainly criticized the quality of the approval study and requested that further studies be carried out before a final decision on approval was made. Lenalidomide has now also been approved in Germany for the treatment of the 5q-minus syndrome.

Individual evidence

  1. ↑ A. List, G. Dewald, J. Bennett, A. Giagounidis, A. Raza, E. Feldman, B. Powell, P. Greenberg, D. Thomas, R. Stone, C. Reeder, K. Wride, J Patin, M. Schmidt, J. Zeldis, R. Knight: Lenalidomide in the myelodysplastic syndrome with chromosome 5q deletion. In: The New England Journal of Medicine . Volume 355, Number 14, October 2006, pp. 1456-1465, doi: 10.1056 / NEJMoa061292 , PMID 17021321 .
  2. ↑ FDA Approves New Treatment for Myelodysplastic Syndrome (MDS). (No longer available online.) US Food and Drug Administration (FDA) December 28, 2005, archived from the original January 1, 2006 ; accessed on February 22, 2013 (English).
  3. ^ New Drugs Online Report for lenalidomide. National Health Service (NHS), accessed February 22, 2013 .