The Chronicles of Myelodysplastic/ Myeloproliferative Legacy - Chronic Myelomonocytic Leukemia
AbstractChronic myelomonocytic leukemia is a heterogeneous syndrome with features of both myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN). The varied clinical presentations add to the distinctiveness of the disease. This heterogeneity should invigorate the search for reliable predictors of evolution and progression of disease. We report a case series from a tertiary care centre in Kerala, South India. This was a retrospective observational study of all cases of CMML, which was diagnosed in the departments of Pathology and Clinical Haematology & Haemato- oncology of our institution between January 2017 to May 2020. The clinical presentation, laboratory investigations, and treatment details were noted. Nine cases of CMML were encountered during the study period. The mean age of study subjects was 70.4 years with a female predilection. Fever and weight loss were the most common clinical presentations. Four patients were classified as CMML- 2, three patients as CMML- 1, and two as CMML- 0. Based on the WBC count, five patients were classified as dysplastic and four as proliferative subtypes. Two patients had grade 1/3 (one case each of CMML- 2 and CMML- 1) and one patient had grade 2/3 fibrosis (a case of CMML- 1) in the bone marrow. Thirty-three percentage patients had clonal cytogenetic abnormalities, the commonest being trisomy 8. Renal function was deranged in three patients and two patients had a deranged liver function and hepatomegaly. Four patients underwent treatment with hypomethylating agents or cytoreduction with hydroxyurea. One of the patients (CMML- 2 with marked leucocytosis) succumbed to disease.
Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391-2405.
Patnaik MM, Parikh SA, Hanson CA, Tefferi A. Chronic myelomonocytic leukaemia: a concise clinical and pathophysiological review. Br J Haematol. 2014;165(3):273-286.
Kohlmann A, Grossmann V, Klein HU, et al. Next-generation sequencing technology reveals a characteristic pattern of molecular mutations in 72.8% of chronic myelomonocytic leukemia by detecting frequent alterations in TET2, CBL, RAS, and RUNX1. J Clin Oncol. 2010;28(24):3858-3865.
Martiat P, Michaux JL, Rodhain J (1991). Philadelphia -negative (Ph-) chronic myeloidleukemia (CML): comparison with Ph+ CML and chronic myelomonocytic leukemia. The Groupe Francais de Cytogenetique Hematologique. Blood. 78:205- 11.
Orazi A, Chiu R, O'Malley DP, et al.(2006). Chronic myelomonocytic leukemia: The role of bone marrow biopsy immunohistology.Mod Pathol. 19:1536-45.
Selimoglu-Buet D, Wagner-Ballon 0, Saada V, et al. (2015). Characteristic repartition of monocyte subsets as a diagnostic signature of chronic myelomonocytic leukemia. Blood. 125:3618-26.
Greenberg P, Cox C, LeBeau MM, et al International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 1997;89(6):2079-2088
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:e333
Hunter AM, Zhang L, Padron E. Current Management and Recent Advances in the Treatment of Chronic Myelomonocytic Leukemia. Curr Treat Options Oncol. 2018;19(12):67. Published 2018 Oct 27. doi:10.1007/s11864-018-0581-6
Nabeel Azeez K , Venkatesan Somasundaram , Isha Sharma , Sanjeevan Sharma and Ajay Malik .Clinicopathological Profile of Chronic Myelomonocytic Leukemia Cases: An Experience from A Tertiary Care Center , Annals of Pathology and Laboratory Medicine, Vol. 6, Issue 10, October, 2019, eISSN: 2349-6983; pISSN: 2394-6466
Patnaik MM, Wassie EA, Padron E, et al. Chronic myelomonocytic leukemia in younger patients: molecular and cytogenetic predictors of survival and treatment outcome [published correction appears in Blood Cancer J. 2015;5:e280] [published correction appears in Blood Cancer J. 2015;5:e280]. Blood Cancer J. 2015;5(1):e270.
Bennett JM, Catovsky D, Daniel MT, et al. Proposals for the classification of the acute leukaemias. French-American-British (FAB) co-operative group. Br JHaematol. 1976;33:451–458
Germing U, Kündgen A, GattermannN. Risk assessment in chronic myelomonocytic leukemia(CMML). Leuk Lymphoma. 2004;45(7):1311‐1318.
Patnaik MM, Tefferi A. Cytogenetic and molecular abnormalities in chronic myelomonocytic leukemia. Blood Cancer J. 2016; 6:e 393.
Petrova-Drus, K., Chiu, A., Margolskee, E., Barouk-Fox, S., Geyer, J., Dogan, A., & Orazi, A. (2017). Bone marrow fibrosis in chronic myelomonocytic leukemia is associated with increased megakaryopoiesis, splenomegaly and with a shorter median time to disease progression. Oncotarget, 8(61), 103274–103282.
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access at http://opcit.eprints.org/oacitation-biblio.html).