Clinicopathological Profile of Chronic Myelomonocytic Leukemia Cases
An Experience from A Tertiary Care Center
Keywords:
Chronic Myelomonocytic Leukemia, Survival, Ring sideroblast, Myelodysplastic syndrome, Myeloproliferative syndrome, Myelodysplastic/myeloproliferative neoplasms, Hematopathology
Abstract
Background: Chronic myelomonocytic leukemia (CMML) is a clonal hematological neoplasm with features of both myeloproliferation and myelodysplasia with an incidence 0.4 per lakh population. A number of prognostic risks scoring systems have attempted to predict survival and risk of CMML patients, like International Prognostic Scoring System (IPSS), the Spanish Score, the modified Bournemouth Score, the Dusseldorf Score and the MDAP Score. But no prognostic system has been widely accepted. More data from different regions are required to create a widely accepted working prognostic system for CMML. No case series on CMML has been published in literature from India in our knowledge. This article attempts to put to light the various clinicopathological parameters of CMML cases from India and the impact of these parameters on final outcome. Methods: All admitted patients in a tertiary center in western India, with a diagnosis of either a chronic myeloproliferative disease or a myelodysplastic disease over a period of 3 years (2015-2018) were evaluated, out of which nine(n=9) cases fulfilled the diagnostic criteria of CMML. All patients underwent peripheral blood examination, bone marrow aspirate, bone marrow biopsy and cytogenetic studies. Result: All patients (n=9) were between 50 and 80 years and most were males (n=8). Five patients presented with hepatosplenomegaly. Renal and liver function of one patient was deranged who had pleural effusion, ascites. Most patients (n=8) had total leukocyte count above 13000/cumm, while three had low platelet counts. Two out of three patients classified as CMML-II with increased blasts in peripheral blood and bone marrow had fatal outcomes. Patients whose karyotypes were available had normal karyotypes without any additional cytogenetic abnormalities. All were negative for JAK2 and BCR-ABL1. Conclusion: The study concluded that altered biochemical tests (LDH, LFT), blast percentage, CMML II, relative lymphocytosis and transformation to AML were associated with poor outcome.References
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11. Such E, Senent L, Nomdedeu B, Bueno J, Bernal T, Carbonell F, Cervera J, Florensa L, del Cañizo MC, Belkaid M et al: Chronic Myelomonocytic Leukemia (CMML) with More Than 15% of Ring Sideroblasts in Bone Marrow: An Overlapping Disorder Between CMML and Refractory Anemia with Ring Sideroblasts. Blood 2009, 114(22):290-290.
12. Such E, Germing U, Malcovati L, Cervera J, Kuendgen A, Della Porta MG, Nomdedeu B, Arenillas L, Luno E, Xicoy B et al: Development and validation of a prognostic scoring system for patients with chronic myelomonocytic leukemia. Blood 2013, 121(15):3005-3015.
13. Yim KH, Nahm FS, Han KA, Park SY: Analysis of statistical methods and errors in the articles published in the korean journal of pain. The Korean journal of pain 2010, 23(1):35-41.
14. Shapiro SS, Wilk MB: An analysis of variance test for normality (complete samples)†. Biometrika 1965, 52(3-4):591-611.
15. Storniolo AM, Moloney WC, Rosenthal DS, Cox C, Bennett JM: Chronic myelomonocytic leukemia. Leukemia 1990, 4(11):766-770.
16. Onida F, Kantarjian HM, Smith TL, Ball G, Keating MJ, Estey EH, Glassman AB, Albitar M, Kwari MI, Beran M: Prognostic factors and scoring systems in chronic myelomonocytic leukemia: a retrospective analysis of 213 patients. Blood 2002, 99(3):840-849.
17. Cortes J: CMML: a biologically distinct myeloproliferative disease. Current hematology reports 2003, 2(3):202-208.
2. Cho S-R, Park JS, Moon Y, Kim MH, Ahn J-Y, Huh J, Kim HR, Han JH: WHO2016 Vs. WHO2008 Criteria for the Diagnosis of Chronic Myelomonocytic Leukemia. Blood 2017, 130(Suppl 1):5314.
3. Alessandrino EP, Orlandi E, Brusamolino E, Lazzarino M, Morra E, Castagnola C, Bernasconi C: Chronic myelomonocytic leukemia: clinical features, cytogenetics, and prognosis in 30 consecutive cases. Hematological oncology 1985, 3(2):147-155.
4. Germing U, Gattermann N, Minning H, Heyll A, Aul C: Problems in the classification of CMML--dysplastic versus proliferative type. Leukemia research 1998, 22(10):871-878.
5. Nösslinger T, Reisner R, Grüner H, Tüchler H, Nowotny H, Pittermann E, Pfeilstöcker M: Dysplastic versus proliferative CMML – a retrospective analysis of 91 patients from a single institution. Leukemia research 2001, 25(9):741-747.
6. Parilla M, Venkataraman G: The thin line between CML and CMML. Blood 2017, 129(17):2456.
7. Harrington AM, Schelling LA, Ordobazari A, Olteanu H, Hosking PR, Kroft SH: Immunophenotypes of Chronic Myelomonocytic Leukemia (CMML) Subtypes by Flow Cytometry A Comparison of CMML-1 vs CMML-2, Myeloproliferative vs Dysplastic, De Novo vs Therapy-Related, and CMML-Specific Cytogenetic Risk Subtypes. American journal of clinical pathology 2016, 146(2):170-181.
8. Patnaik MM, Tefferi A: Cytogenetic and molecular abnormalities in chronic myelomonocytic leukemia. Blood cancer journal 2016, 6:e393.
9. Germing U, Kündgen A, Gattermann N: Risk Assessment in Chronic Myelomonocytic Leukemia (CMML). Leukemia & Lymphoma 2004, 45(7):1311-1318.
10. Gelsi-Boyer V, Cervera N, Bertucci F, Brecqueville M, Finetti P, Murati A, Arnoulet C, Mozziconacci M-J, Mills KI, Cross NCP et al: Molecular similarity between myelodysplastic form of chronic myelomonocytic leukemia and refractory anemia with ring sideroblasts. Haematologica 2013, 98(4):576-583.
11. Such E, Senent L, Nomdedeu B, Bueno J, Bernal T, Carbonell F, Cervera J, Florensa L, del Cañizo MC, Belkaid M et al: Chronic Myelomonocytic Leukemia (CMML) with More Than 15% of Ring Sideroblasts in Bone Marrow: An Overlapping Disorder Between CMML and Refractory Anemia with Ring Sideroblasts. Blood 2009, 114(22):290-290.
12. Such E, Germing U, Malcovati L, Cervera J, Kuendgen A, Della Porta MG, Nomdedeu B, Arenillas L, Luno E, Xicoy B et al: Development and validation of a prognostic scoring system for patients with chronic myelomonocytic leukemia. Blood 2013, 121(15):3005-3015.
13. Yim KH, Nahm FS, Han KA, Park SY: Analysis of statistical methods and errors in the articles published in the korean journal of pain. The Korean journal of pain 2010, 23(1):35-41.
14. Shapiro SS, Wilk MB: An analysis of variance test for normality (complete samples)†. Biometrika 1965, 52(3-4):591-611.
15. Storniolo AM, Moloney WC, Rosenthal DS, Cox C, Bennett JM: Chronic myelomonocytic leukemia. Leukemia 1990, 4(11):766-770.
16. Onida F, Kantarjian HM, Smith TL, Ball G, Keating MJ, Estey EH, Glassman AB, Albitar M, Kwari MI, Beran M: Prognostic factors and scoring systems in chronic myelomonocytic leukemia: a retrospective analysis of 213 patients. Blood 2002, 99(3):840-849.
17. Cortes J: CMML: a biologically distinct myeloproliferative disease. Current hematology reports 2003, 2(3):202-208.
Published
2019-11-01
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