Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Letter to the Editor
  • Published:

Dysgranulopoiesis is an independent adverse prognostic factor in chronic myeloid disorders with an isolated interstitial deletion of chromosome 5q

Leukemia volume 23, pages 796–800 (2009)Cite this article

Myelodysplastic syndromes (MDS) are acquired clonal bone marrow disorders characterized by peripheral blood cytopenias and abnormal bone marrow hematopoiesis. An interstitial deletion of the long arm of chromosome 5 [del(5q)], either in isolation or associated with other chromosomal abnormalities, is the most frequent karyotypic abnormality identified in MDS. A distinct subset of MDS with isolated del(5q) was recognized first by Van den Berghe et al.1 and later by others2, 3, 4, 5 owing to its unique clinical and pathologic features (‘5q− features’), which included a female preponderance, macrocytic anemia, frequent thrombocytosis, monolobated megakaryocytes and favorable clinical outcomes. This clinical entity was subsequently renamed by the 2001 World Health Organization (WHO) Classification of Hematopoietic Neoplasms6 as ‘5q− syndrome’ and required only the presence of an isolated del(5q) and less than 5% bone marrow blasts. This definition was primarily based on several studies2, 3, 4 that showed the adverse prognostic impact of blast count in peripheral blood or bone marrow.

However, the WHO classification did not address two issues: first, the prototypic clinical and pathologic ‘5q− features,’ which are not a prerequisite in the WHO diagnostic criteria, are frequently absent in cases that fulfill the WHO definition of ‘5q− syndrome’. The prognostic significance of the prototypic ‘5q− features’ is unclear. Second, the prognostic impact of dysgranulopoiesis in the 5q− syndrome was not addressed. Both issues are frequently encountered in clinical practice but have remained unanswered in the literature.

This is a preview of subscription content, access via your institution

Relevant articles

Open Access articles citing this article.

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1

References

  1. Van den Berghe H, Cassiman JJ, David G, Fryns JP, Michaux JL, Sokal G . Distinct haematological disorder with deletion of long arm of no. 5 chromosome. Nature 1974; 251: 437–438.

    Article  CAS  PubMed  Google Scholar 

  2. Dewald GW, Davis MP, Pierre RV, O'Fallon JR, Hoagland HC . Clinical characteristics and prognosis of 50 patients with a myeloproliferative syndrome and deletion of part of the long arm of chromosome 5. Blood 1985; 66: 189–197.

    CAS  PubMed  Google Scholar 

  3. Boultwood J, Lewis S, Wainscoat JS . The 5q− syndrome. Blood 1994; 84: 3253–3260.

    CAS  PubMed  Google Scholar 

  4. Mathew P, Tefferi A, Dewald GW, Goldberg SL, Su J, Hoagland HC et al. The 5q− syndrome: a single-institution study of 43 consecutive patients. Blood 1993; 81: 1040–1045.

    CAS  PubMed  Google Scholar 

  5. Giagounidis AA, Germing U, Haase S, Hildebrandt B, Schlegelberger B, Schoch C et al. Clinical, morphological, cytogenetic, and prognostic features of patients with myelodysplastic syndromes and del(5q) including band q31. Leukemia 2004; 18: 113–119.

    Article  CAS  PubMed  Google Scholar 

  6. Brunning RD, Bennett JM, Flandrin G, Matutes E, Head D, Vardiman JW et al. Myelodysplastic syndrome associated with isolated del(5q) chromosome abnormality (‘5q− syndrome’). Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues: WHO Classification of Tumours. IARC Press: Lyons, France, 2001, pp 73–73.

    Google Scholar 

  7. Seo IS, Li CY, Yam LT . Myelodysplastic syndrome: diagnostic implications of cytochemical and immunocytochemical studies. Mayo Clin Proc 1993; 68: 47–53.

    Article  CAS  PubMed  Google Scholar 

  8. Santana-Davila R, Tefferi A, Holtan SG, Ketterling RP, Dewald GW, Knudson RA et al. Primary myelofibrosis is the most frequent myeloproliferative neoplasm associated with del(5q): Clinicopathologic comparison of del(5q)-positive and -negative cases. Leuk Res 2008; 32: 1927–1930.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Hematopathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA

    D Chen, J D Hoyer & C A Hanson

  2. Division of Laboratory Genetics, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA

    R P Ketterling & G W Dewald

  3. Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA

    A Tefferi, D P Steensma, S G Holtan, R Santana-Davila & L F Porrata

Authors
  1. D Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. J D Hoyer
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. R P Ketterling
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. A Tefferi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. D P Steensma
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. S G Holtan
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. R Santana-Davila
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. L F Porrata
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. G W Dewald
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. C A Hanson
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to D Chen.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Chen, D., Hoyer, J., Ketterling, R. et al. Dysgranulopoiesis is an independent adverse prognostic factor in chronic myeloid disorders with an isolated interstitial deletion of chromosome 5q. Leukemia 23, 796–800 (2009). https://doi.org/10.1038/leu.2008.279

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/leu.2008.279

This article is cited by

Search

Advanced search

Quick links