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Fang H, Yabe M, Zhang X, et al. Myelodysplastic syndrome with t(6;9)(p22;q34.1)/DEK-NUP214 better classified as acute myeloid leukemia? A multicenter study of 107 cases. Mod Pathol. 2021;34(6):1143-1152doi: 10.1038/s41379-021-00741-w.
Fang, H., Yabe, M., Zhang, X., Kim, Y., Wu, X., Wei, P., Chi, S., Zheng, L., Garcia-Manero, G., Shao, L., Yuan, J., Shen, Y., Zheng, G., Tang, G., Wang, W., Loghavi, S., Shen, Q., Yuan, Y., He, R., Chen, D., Medeiros, L. J., & Hu, S. (2021). Myelodysplastic syndrome with t(6;9)(p22;q34.1)/DEK-NUP214 better classified as acute myeloid leukemia? A multicenter study of 107 cases. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 34(6), 1143-1152. https://doi.org/10.1038/s41379-021-00741-w
Fang, Hong, et al. "Myelodysplastic syndrome with t(6;9)(p22;q34.1)/DEK-NUP214 better classified as acute myeloid leukemia? A multicenter study of 107 cases." Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc vol. 34,6 (2021): 1143-1152. doi: https://doi.org/10.1038/s41379-021-00741-w
Fang H, Yabe M, Zhang X, Kim Y, Wu X, Wei P, Chi S, Zheng L, Garcia-Manero G, Shao L, Yuan J, Shen Y, Zheng G, Tang G, Wang W, Loghavi S, Shen Q, Yuan Y, He R, Chen D, Medeiros LJ, Hu S. Myelodysplastic syndrome with t(6;9)(p22;q34.1)/DEK-NUP214 better classified as acute myeloid leukemia? A multicenter study of 107 cases. Mod Pathol. 2021 Jun;34(6):1143-1152. doi: 10.1038/s41379-021-00741-w. Epub 2021 Feb 08. PMID: 33558656.
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Mod Pathol. 2021 Jun;34(6):1143-1152. doi: 10.1038/s41379-021-00741-w. Epub 2021 Feb 08.

Myelodysplastic syndrome with t(6;9)(p22;q34.1)/DEK-NUP214 better classified as acute myeloid leukemia? A multicenter study of 107 cases.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc

Hong Fang, Mariko Yabe, Xiaohui Zhang, Young Kim, Xiaojun Wu, Peng Wei, Sunyi Chi, Lan Zheng, Guillermo Garcia-Manero, Lina Shao, Ji Yuan, Yulei Shen, Gang Zheng, Guiling Tang, Wei Wang, Sanam Loghavi, Qi Shen, Yongzhong Yuan, Rong He, Dong Chen, L Jeffrey Medeiros, Shimin Hu

Affiliations

  1. Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
  2. Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
  3. Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.
  4. Department of Pathology, City of Hope National Medical Center, Duarte, CA, USA.
  5. Department of Pathology, John Hopkins University, Baltimore, MD, USA.
  6. Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
  7. Quantitative Sciences Program, The University of Texas MD Anderson Cancer Center/UTHealth Graduate School of Biomedical Sciences, Houston, TX, USA.
  8. Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
  9. Department of Pathology, The University of Michigan Medical Center, Ann Arbor, MI, USA.
  10. Department of Pathology, University of Nebraska Medical Center, Omaha, NE, USA.
  11. Department of Pathology, Advent Health-Orlando, Orlando, FL, USA.
  12. Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AK, USA.
  13. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
  14. Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. [email protected].

PMID: 33558656 DOI: 10.1038/s41379-021-00741-w

Abstract

t(6;9)(p22;q34.1)/DEK-NUP214 is a recurrent genetic abnormality that occurs in 1-2% of patients with acute myeloid leukemia (AML), and rarely in myelodysplastic syndrome (MDS). It has been suggested by others that all myeloid neoplasms with t(6;9)/DEK-NUP214 may be considered as AML, even when blast count is <20%. In this study, we compared the clinicopathologic features of 107 patients with myeloid neoplasms harboring t(6;9)/DEK-NUP214: 33 MDS and 74 AML. Compared with patients with AML, patients with MDS were older (p = 0.10), had a lower white blood cell count (p = 0.0017), a lower blast count in the peripheral blood (p < 0.0001) and bone marrow (p < 0.0001), a higher platelet count (p = 0.022), and a lower frequency of FLT3-ITD mutation (p = 0.01). In addition, basophilia was not a common feature in the patients of this cohort. Although there was no difference in overall survival between MDS and AML patients (p = 0.18) in the entire cohort, the survival curves did show a trend toward favorable survival in MDS patients. Multivariate analyses showed that initial diagnosis of MDS vs. AML and allogeneic hematopoietic stem cell transplantation were prognostic factors for survival of patients with t(6;9)/DEK-NUP214 (p = 0.008 and p < 0.0001, respectively). Our data suggest that MDS with t(6;9)/DEK-NUP214 is prognostically not equivalent to AML with t(6;9)/DEK-NUP214. These data also show that stem cell transplantation greatly improves the survival of MDS and AML patients with myeloid neoplasms associated with t(6;9)/DEK-NUP214.

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