Skip to main content
Log in

Prognostic assessment for chronic myelomonocytic leukemia in the context of the World Health Organization 2016 proposal: a multicenter study of 280 patients

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Knowledge on chronic myelomonocytic leukemia (CMML) patients from Argentina and Brazil is limited. Our series of 280 patients depicted an older age at diagnosis (median 72 years old), 26% of aberrant karyotypes, and a prevalence of myelodysplastic (60%) and CMML-0 subtypes (56%). The median overall survival (OS) was 48.2 months for patients in CMML-0 (Ref.), 24.7 months for those in CMML-1 (HR = 2.0, p = 0.001), and 8.8 months for patients in CMML-2 (HR = 4.6, p < 0.001). In the CMML-0 category, median OS were different between myelodysplastic and myeloproliferative subtypes (63.7 vs 21.2 months, p < 0.001); however, no differences were observed within CMML-1 and CMML-2 subtypes (24.7 vs 23.7 months, p = 0.540, and 9.1 vs 8.2 months, p = 0.160). The prognostic impact of 24 variables and 7 prognostic systems was adjusted to the WHO 2016 after validating their usefulness. Multivariate analysis were performed, and the final model revealed Hb ≥ 8 -< 10g/dL (HR 1.7), Hb < 8g/dL (HR 2.8), poor karyotypes (HR 2.1), WHO 2016-CMML-1 (HR 2.1), and CMML-2 (HR 3.5) as independent adverse clinical parameters in our cohort with a borderline influence of platelets count < 50 × 109/L (HR 1.4). We could validate several scoring systems, the WHO 2016 proposal and its prognostic capability, along with accessible covariates, on predicting the outcome in our series of CMML patients from Latin America.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Arber D, Orazi A, Hasserjian R et al (2016) The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 127:2391–2406

    Article  CAS  Google Scholar 

  2. Patnaik M, Tefferi A, Lanier W et al (2016) Chronic myelomonocytic leukemia: focus on clinical practice. Mayo Clin Proc 91:259–272

    Article  Google Scholar 

  3. Neuwirtová R, Mociková K, Musilová J et al (1997) Mixed myelodysplastic and myeloproliferative syndromes. Cas Lek Cesk 136:724–729

    PubMed  Google Scholar 

  4. Vardiman JW, Harris NL, Brunning R (2002) The World Health Organization (WHO) classification of the myeloid neoplasms. Blood 100:2292–2302

    Article  CAS  Google Scholar 

  5. Schuler E, Schroeder M, Neukirchen J, Strupp C, Xicoy B, Kündgen A, Hildebrandt B, Haas R, Gattermann N, Germing U (2014) Refined medullary blast and white blood cell count based classification of chronic myelomonocytic leukemias. Leuk Res 38:1413–1419

    Article  CAS  Google Scholar 

  6. Worsley A, Oscier DG, Stevens J, Darlow S, Figes A, Mufti GJ, Hamblin TJ (1988) Prognostic features of chronic myelomonocytic leukaemia: a modified Bournemouth score gives the best prediction of survival. Br J Haematol 68:17–21

    Article  CAS  Google Scholar 

  7. Germing U, Kündgen A, Gattermann N (2004) Risk assessment in chronic myelomonocytic leukemia (CMML). Leuk Lymphoma 45:1311–1318

    Article  CAS  Google Scholar 

  8. Onida FE, Kantarjian HM, Smith TL, Ball G, Keating MJ, Estey EH, Glassman AB, Albitar M, Kwari MI, Beran M (2002) Prognostic factors and scoring systems in chronic myelomonocytic leukemia: a retrospective analysis of 213 patients. Blood 99:840–849

    Article  CAS  Google Scholar 

  9. Such E, Germing U, Malcovati L, Cervera J, Kuendgen A, Della Porta MG, Nomdedeu B, Arenillas L, Luño E, Xicoy B, Amigo ML, Valcarcel D, Nachtkamp K, Ambaglio I, Hildebrandt B, Lorenzo I, Cazzola M, Sanz G (2013) Development and validation of a prognostic scoring system for patients with chronic myelomonocytic leukemia. Blood 121:3005–3015

    Article  CAS  Google Scholar 

  10. Padron E, Garcia Manero G, Patnaik M et al (2015) An international data set for CMML validates prognostic scoring systems and demonstrates a need for novel prognostication strategies. Blood Cancer J 5:e333

    Article  CAS  Google Scholar 

  11. Loghavi S, Sui D, Wei P et al (2018) Validation of the 2017 revision of the WHO chronic myelomonocytic leukemia categories. Blood Adv 28:1807–1816

    Article  Google Scholar 

  12. Greenberg P, Cox C, LeBeau MM et al (1997) International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 89:2079–2088

    Article  CAS  Google Scholar 

  13. Greenberg PL, Tuechler H, Schanz J, Sanz G, Garcia-Manero G, Solé F, Bennett JM, Bowen D, Fenaux P, Dreyfus F, Kantarjian H, Kuendgen A, Levis A, Malcovati L, Cazzola M, Cermak J, Fonatsch C, le Beau MM, Slovak ML, Krieger O, Luebbert M, Maciejewski J, Magalhaes SMM, Miyazaki Y, Pfeilstöcker M, Sekeres M, Sperr WR, Stauder R, Tauro S, Valent P, Vallespi T, van de Loosdrecht AA, Germing U, Haase D (2012) Revised international prognostic scoring system for myelodysplastic syndromes. Blood 120:2454–2465

    Article  CAS  Google Scholar 

  14. Zeidan AM, Sekeres MA, Wang XF, al Ali N, Garcia-Manero G, Steensma DP, Roboz G, Barnard J, Padron E, DeZern A, Maciejewski JP, List AF, Komrokji RS, on Behalf of the MDS Clinical Research Consortium (Funded by Edward P. Evans Foundation) (2015) Comparing the prognostic value of risk stratifying models for patients with lower-risk myelodysplastic syndromes: is one model better? Am J Hematol 90:1036–1040

    Article  Google Scholar 

  15. Onida F (2017) Models of prognostication in chronic myelomonocytic leukemia. Curr Hematol Malig Rep 12:513–521

    Article  Google Scholar 

  16. Belli CB, Pinheiro RF, Bestach Y, Larripa IB, da Silva Tanizawa RS, Alfonso G, Gonzalez J, Rosenhain M, Watman N, Cavalcante de Andrade Silva M, Negri Aranguren P, García Rivello H, Magalhaes SMM, Valladares X, Undurraga MS, Velloso ERP (2015) Myelodysplastic syndromes in South America: a multinational study of 1080 patients. Am J Hematol 90:851–858

    Article  CAS  Google Scholar 

  17. Rollison DE, Howlader N, Smith MT, Strom SS, Merritt WD, Ries LA, Edwards BK, List AF (2008) Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001-2004, using data from the NAACCR and SEER programs. Blood 112:45–52

    Article  CAS  Google Scholar 

  18. Elena C, Galli A, Such E et al (2016) Integrating clinical features and genetic lesions in the risk assessment of patients with chronic myelomonocytic leukemia. Blood 128:1408–1418

    Article  CAS  Google Scholar 

  19. Tang MG, Zhang L, Fu B et al (2014) Cytogenetic risk stratification of 417 patients with chronic myelomonocytic leukemia from a single institution. Am J Hematol 89:813–818

    Article  Google Scholar 

  20. Beran M, Wen S, Shen Y, Onida F, Jelinek J, Cortes J, Giles F, Kantarjian H (2007) Prognostic factors and risk assessment in chronic myelomonocytic leukemia: validation study of the M.D. Anderson Prognostic Scoring System. Leuk Lymphoma 48:1150–1160

    Article  Google Scholar 

  21. Alfonso A, Montalban-Bravo G, Garcia-Manero G (2017) Current management of patients with chronic myelomonocytic leukemia. Curr Opin Oncol 29:79–87

    Article  CAS  Google Scholar 

  22. Such E, Cervera J, Costa D, Sole F, Vallespi T, Luno E, Collado R, Calasanz MJ, Hernandez-Rivas JM, Cigudosa JC, Nomdedeu B, Mallo M, Carbonell F, Bueno J, Ardanaz MT, Ramos F, Tormo M, Sancho-Tello R, Canizo C, Gomez V, Marco V, Xicoy B, Bonanad S, Pedro C, Bernal T, Sanz GF (2011) Cytogenetic risk stratification in chronic myelomonocytic leukemia. Haematologica 96:375–383

    Article  Google Scholar 

  23. Itzykson R, Kosmider O, Renneville A, Gelsi-Boyer V, Meggendorfer M, Morabito M, Berthon C, Adès L, Fenaux P, Beyne-Rauzy O, Vey N, Braun T, Haferlach T, Dreyfus F, Cross NCP, Preudhomme C, Bernard OA, Fontenay M, Vainchenker W, Schnittger S, Birnbaum D, Droin N, Solary E (2013) Prognostic score including gene mutations in chronic myelomonocytic leukemia. J Clin Oncol 31:2428–2436

    Article  CAS  Google Scholar 

  24. Xicoy B, Triguero A, Such E, García O, Jiménez MJ, Arnán M, Bernal T, Diaz-Beya M, Valcárcel D, Pedro C, Ramos F, Amigo ML, Collado R, Palomo L, Ardanaz MT, Cedena MT, Grau J, Zamora L, Sanz G (2018) The division of chronic myelomonocytic leukemia (CMML)-1 into CMML-0 and CMML-1 according to 2016 World Health Organization (WHO) classification has no impact in outcome in a large series of patients from the Spanish group of MDS. Leuk Res 70:34–36

    Article  Google Scholar 

  25. Solary E, Itzykson R (2017) How I treat chronic myelomonocytic leukemia. Blood 130:126–133

    Article  CAS  Google Scholar 

  26. Moyo TK, Savona MR (2017) Therapy for chronic myelomonocytic leukemia in a new era. Curr Hematol Malig Rep 12:468–477

    Article  Google Scholar 

Download references

Acknowledgements

On behalf of the Grupo de Estudio de SMD, Sociedad Argentina de Hematología, Buenos Aires, Argentina, and the Grupo Latinoamericano de Mielodisplasia (GLAM), the authors thank the investigators of the Argentinean MDS’s Study Group organized by the Argentinean Society of Hematology for the use of the MDS Registry database.

Availability of data and materials

The dataset generated and analyzed during the current study are available from the corresponding author on request.

Funding

This work was not specifically supported, but CB receives grants from the Agencia Nacional de Promoción Científica y Tecnológica (ANPCyT) [PICT 0480 and PICT 1623] and from the Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET) [PIP 0056].

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Jacqueline S. González.

Ethics declarations

Ethics approval statement

All procedures performed in studies involving the patient were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The study was approved by the Ethics Committee of the Institutos de la Academia Nacional de Medicina. Informed consent was obtained according to institutional guidelines.

Conflict of interest

The 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

ESM 1

(PDF 297 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

González, J.S., Perusini, M.A., Basquiera, A.L. et al. Prognostic assessment for chronic myelomonocytic leukemia in the context of the World Health Organization 2016 proposal: a multicenter study of 280 patients. Ann Hematol 100, 1439–1449 (2021). https://doi.org/10.1007/s00277-021-04539-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-021-04539-3

Keywords

Navigation