CC BY 4.0 · Indian J Med Paediatr Oncol 2023; 44(04): 422-427
DOI: 10.1055/s-0043-1766130
Original Article

An Assessment of the Three Popular Prognostic Scoring Systems for Chronic Myelomonocytic Leukemia (CMML) in an Indian Context

1   Department of Oncopathology, Gujarat Cancer and Research Institute, Asarwa, Ahmedabad, Gujarat, India
,
1   Department of Oncopathology, Gujarat Cancer and Research Institute, Asarwa, Ahmedabad, Gujarat, India
,
2   Department of Pathology, Banas Medical College and Research Institute, Palanpur, Gujarat, India
,
1   Department of Oncopathology, Gujarat Cancer and Research Institute, Asarwa, Ahmedabad, Gujarat, India
,
1   Department of Oncopathology, Gujarat Cancer and Research Institute, Asarwa, Ahmedabad, Gujarat, India
,
1   Department of Oncopathology, Gujarat Cancer and Research Institute, Asarwa, Ahmedabad, Gujarat, India
› Author Affiliations

Abstract

Introduction: Chronic myelomonocytic leukemia (CMML) is a rare clonal hematopoietic neoplasm with a prevalence of 1.05 to 1.94 cases per 1,00,000 population. There are multiple prognostic scoring system used in practice for CMML, which include both cytogenetic and next-generation sequencing based.

Objective This study assesses the clinicohematological profile of CMML patients, along with comparison of three widely used prognostic scoring systems for CMML (CMML-specific prognostic scoring system, MD Anderson prognostic score, Mayo prognostic model).

Materials and Methods: This study is an 8-year retrospective study. All relevant data had been retrieved and reviewed by the authors. Inclusion and exclusion criteria: All the cases that were diagnosed before 2016 as per 2008 criteria were reclassified, (2) all the cases that were positive for the mutations associated with myeloproliferative neoplasms were excluded, and (3) cases with more than or equal to 20% blast/blast equivalents were excluded. A univariate analysis was done followed by a multivariate analysis for all the parameters constituting each scoring system. Lastly, a receiver operating characteristic curve was plotted for all the three scoring systems.

Result: There were total 23 patients, with a median age of 63 years and a male to female ratio of 2.3:1. Cytogenetic aberration and genetic mutation were observed in 6 and 3 cases, respectively. The median overall survival (OS) was 48 months and the median leukemia-free survival was 12 months. Post-multivariate analysis, the parameters with significant impact on OS were absolute monocyte count more than 10 × 10^9/L, myeloid precursors in peripheral blood, hemoglobin less than 10g/dL, platelet less than 100 × 10^9/L, hemoglobin less than 12g/dL, and absolute lymphocyte count more than 2.5 × 10^9/L.

Conclusion: To summarize, we discovered CPSS to be a better prognostic tool for a setup like ours, since molecular investigations are not always readily available for each case. More such researches are needed in the near future so that we can design better prognostic tools and see for their usefulness in real life.



Publication History

Article published online:
17 April 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Orazi A, Benett JM, Germing U. et al. Chronic myelomonocytic leukemia. In: Swerdlow SH, Campo E, Harris NL. et al., eds. WHO Classification of Tumors of Haematopoetic and Lymphoid tissues. 4th ed. Lyon: International agency for research on cancer (IARC); 2017: 82-86
  • 2 Patnaik MM, Tefferi A. Chronic myelomonocytic leukemia: 2020 update on diagnosis, risk stratification and management. Am J Hematol 2020; 95 (01) 97-115
  • 3 Elena C, Gallì A, Such E. et al. Integrating clinical features and genetic lesions in the risk assessment of patients with chronic myelomonocytic leukemia. Blood 2016; 128 (10) 1408-1417
  • 4 Such E, Cervera J, Costa D. et al. Cytogenetic risk stratification in chronic myelomonocytic leukemia. Haematologica 2011; 96 (03) 375-383
  • 5 Onida F, Kantarjian HM, Smith TL. et al. Prognostic factors and scoring systems in chronic myelomonocytic leukemia: a retrospective analysis of 213 patients. Blood 2002; 99 (03) 840-849
  • 6 Patnaik MM, Padron E, LaBorde RR. et al. Mayo prognostic model for WHO-defined chronic myelomonocytic leukemia: ASXL1 and spliceosome component mutations and outcomes. Leukemia 2013; 27 (07) 1504-1510 Erratum in: Leukemia. 2013 Oct;27(10):2112. Komroji, R S [corrected to Komrokji, R S]. PMID: 23531518
  • 7 Malcovati L, Germing U, Kuendgen A. et al. Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. J Clin Oncol 2007; 25 (23) 3503-3510
  • 8 Guru Murthy GS, Dhakal I, Mehta P. Incidence and survival outcomes of chronic myelomonocytic leukemia in the United States. Leuk Lymphoma 2017; 58 (07) 1648-1654
  • 9 Calvo X, Nomdedeu M, Santacruz R. et al. Comparison of three prognostic scoring systems in a series of 146 cases of chronic myelomonocytic leukemia (CMML): MD Anderson prognostic score (MDAPS), CMML-specific prognostic scoring system (CPSS) and Mayo prognostic model. A detailed review of prognostic factors in CMML. Leuk Res 2015; S0145-2126 (15)30324–6
  • 10 Hoversten K, Vallapureddy R, Lasho T. et al. Nonhepatosplenic extramedullary manifestations of chronic myelomonocytic leukemia: clinical, molecular and prognostic correlates. Leuk Lymphoma 2018; 59 (12) 2998-3001
  • 11 Azeez N, Somasundaram V, Sharma I, Sharma S, Malik A. Clinicopathological profile of chronic myelomonocytic leukemia cases: an experience from a tertiary care center. APLM 2019; 6 (10) 525-530
  • 12 Padron E, Garcia-Manero G, Patnaik MM. et al. An international data set for CMML validates prognostic scoring systems and demonstrates a need for novel prognostication strategies. Blood Cancer J 2015; 5 (07) e333
  • 13 Roman D, Arenillas L, Parraga I. et al. Generation of a new prognostic index for chronic myelomonocytic leukemia (CMML) based on peripheral blood assessment. Blood 2019; 134 (Suppl. 01) 637
  • 14 Greenberg PL, Tuechler H, Schanz J. et al. Revised international prognostic scoring system for myelodysplastic syndromes. Blood 2012; 120 (12) 2454-2465
  • 15 Patnaik MM, Itzykson R, Lasho TL. et al. ASXL1 and SETBP1 mutations and their prognostic contribution in chronic myelomonocytic leukemia: a two-center study of 466 patients. Leukemia 2014; 28 (11) 2206-2212
  • 16 Padron E. Chronic myelomonocytic leukemia: Management and prognosis. In: Uptodate 2020. Accessed February 22, 2023 at: http://www.uptodate.com/contents/ChronicmyelomonocyticleukemiaonSept04,2020