Skip to main content

Thank you for visiting 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.


Differential prognostic impact of IDH1 and IDH2 mutations in chronic myelomonocytic leukemia

Your institute does not have access to this article

Access options

Buy article

Get time limited or full article access on ReadCube.


All prices are NET prices.

Fig. 1: Overall and AML-free survival outcomes of IDH1/2 mutated CMML patients, along with detailed phenotypic features of TET2 and IDH co-mutated CMML patients.


  1. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127:2391–405.

    CAS  Article  Google Scholar 

  2. Mason CC, Khorashad JS, Tantravahi SK, Kelley TW, Zabriskie MS, Yan D, et al. Age-related mutations and chronic myelomonocytic leukemia. Leukemia. 2016;30:906–13.

    CAS  Article  Google Scholar 

  3. Itzykson R, Solary E. An evolutionary perspective on chronic myelomonocytic leukemia. Leukemia. 2013;27:1441–50.

    CAS  Article  Google Scholar 

  4. Carr RM, Vorobyev D, Lasho T, Marks DL, Tolosa EJ, Vedder A, et al. RAS mutations drive proliferative chronic myelomonocytic leukemia via a KMT2A-PLK1 axis. Nat Commun. 2021;12:2901.

    CAS  Article  Google Scholar 

  5. Patnaik MM, Itzykson R, Lasho TL, Kosmider O, Finke CM, Hanson CA, et al. ASXL1 and SETBP1 mutations and their prognostic contribution in chronic myelomonocytic leukemia: a two-center study of 466 patients. Leukemia. 2014;28:2206–12.

    CAS  Article  Google Scholar 

  6. Ward PS, Patel J, Wise DR, Abdel-Wahab O, Bennett BD, Coller HA, et al. The common feature of leukemia-associated IDH1 and IDH2 mutations is a neomorphic enzyme activity converting alpha-ketoglutarate to 2-hydroxyglutarate. Cancer Cell. 2010;17:225–34.

    CAS  Article  Google Scholar 

  7. Coltro G, Lasho TL, Finke CM, Gangat N, Pardanani A, Tefferi A, et al. Germline SH2B3 pathogenic variant associated with myelodysplastic syndrome/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis. Am J Hematol. 2019;94:E231–E234.

    Article  Google Scholar 

  8. DiNardo CD, Stein EM, de Botton S, Roboz GJ, Altman JK, Mims AS, et al. Durable remissions with ivosidenib in IDH1-mutated relapsed or refractory AML. N Engl J Med. 2018;378:2386–98.

    CAS  Article  Google Scholar 

  9. Patnaik MM, Hanson CA, Hodnefield JM, Lasho TL, Finke CM, Knudson RA, et al. Differential prognostic effect of IDH1 versus IDH2 mutations in myelodysplastic syndromes: a Mayo Clinic study of 277 patients. Leukemia. 2012;26:101–5.

    CAS  Article  Google Scholar 

  10. Savona MR, Malcovati L, Komrokji R, Tiu RV, Mughal TI, Orazi A, et al. An international consortium proposal of uniform response criteria for myelodysplastic/myeloproliferative neoplasms (MDS/MPN) in adults. Blood. 2015;125:1857–65.

    CAS  Article  Google Scholar 

  11. Stein EM, DiNardo CD, Fathi AT, Pollyea DA, Stone RM, Altman JK, et al. Molecular remission and response patterns in patients with mutant-IDH2 acute myeloid leukemia treated with enasidenib. Blood. 2019;133:676–87.

    CAS  Article  Google Scholar 

  12. DiNardo CD, Jabbour E, Ravandi F, Takahashi K, Daver N, Routbort M, et al. IDH1 and IDH2 mutations in myelodysplastic syndromes and role in disease progression. Leukemia. 2016;30:980–4.

    CAS  Article  Google Scholar 

  13. Elena C, Galli A, Such E, Meggendorfer M, Germing U, Rizzo E, et al. Integrating clinical features and genetic lesions in the risk assessment of patients with chronic myelomonocytic leukemia. Blood. 2016;128:1408–17.

    CAS  Article  Google Scholar 

  14. Coltro G, Mangaonkar AA, Lasho TL, Finke CM, Pophali P, Carr R, et al. Clinical, molecular, and prognostic correlates of number, type, and functional localization of TET2 mutations in chronic myelomonocytic leukemia (CMML)-a study of 1084 patients. Leukemia. 2020;34:1407–21.

  15. Coston T, Pophali P, Vallapureddy R, Lasho TL, Finke CM, Ketterling RP, et al. Suboptimal response rates to hypomethylating agent therapy in chronic myelomonocytic leukemia; a single institutional study of 121 patients. Am J Hematol. 2019;94:767–79.

Download references


We would like to acknowledge the “Henry J. Predolin Leukemia Foundation” at Mayo Clinic for supporting the CMML biobank and all the patients at both institutions for participating in research studies.

Author information

Authors and Affiliations



CW and AH helped with data collection and analysis. TL and CF helped with sequencing. MH helped with pathology review. NAA helped with data statistics. AT, NG, AM, RK, EP and MP helped with manuscript writing and editing.

Corresponding authors

Correspondence to Eric Padron or Mrinal M. Patnaik.

Ethics declarations

Competing interests

MP has received research funding from Kura Oncology and Stem Line Pharmaceuticals. The remaining authors declare no competing interests.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary information

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Walsh, C., Hunter, A., Lasho, T. et al. Differential prognostic impact of IDH1 and IDH2 mutations in chronic myelomonocytic leukemia. Leukemia 36, 1693–1696 (2022).

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI:


Quick links