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DOI: 10.4103/ijmpo.ijmpo_62_17
The Biology of Chronic Myelogenous Leukemia in Childhood and Young Adolescents: An Indian Perspective
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Abstract
Objective: The purpose of this study was to determine the clinical, biological, and molecular characteristics at diagnosis in children and adolescents with chronic myelogenous leukemia (CML) in the Indian scenario at our tertiary patient care center. Subjects and Methods: We evaluated 51 children and adolescents with CML registered at our clinic, from January 2007 to December 2015. The mean and median of various parameters were calculated using a Microsoft excel sheet and SPSS software version 16. Results: The median age of presentation in children was 16 years; 92.2% of them were older than 10 years, with a higher prevalence in boys than girls (gender ratio 2.6:1). The symptoms at presentation were fatigue, fever, awareness of mass due to splenomegaly, and bleeding manifestations. One patient presented with Bell's palsy. Markedly raised leukocyte counts were present in 29.4% patients (median white blood cell count >400 × 109/L). Most of the patients presented in the chronic phase of the disease, four each were in accelerated phase and blast crisis, respectively. Majority of patients were categorized as intermediate risk as per Sokal and Hansford score. About 60.7% of these pediatric patients fell in low-risk category as per European Treatment and Outcome Study score at baseline. A predominance of transcript P210-b3a2 (68%) was observed in the children who were studied for the type of chimeric BCR-ABL mRNA. Conclusions: This is one of the most recent reported series of CML in children and adolescents from India highlighting the difference in presentation from adults; mainly hepatomegaly, bleeding manifestations, and higher leukocyte count. Presence of b3a3 transcript of p210 breakpoint of BCR-ABL was more common in children (68%) than b2a2 transcript (32%) when compared to adults as recently described in a study from India, which may explain the differences at presentation.
Publikationsverlauf
Artikel online veröffentlicht:
23. Juni 2021
© 2018. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)
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References
- 1 Ries LAG, Smith MA, Gurney JG, Linet M, Tamra T, Young JL. et al Cancer Incidence and Survival among Children and Adolescents: United States SEER Program 1975-1995, NationalInstitute, SEER Program. NIH Pub. No.99-4649. Bethesda, MD, 1999
- 2 Dikshit RP, Nagrani R, Yeole B, Koyande S, Banawali S. Changing trends of chronic myeloid leukemia in greater Mumbai, India over a period of 30 years. Indian J Med Paediatr Oncol 2011; 32: 96-100
- 3 Vardiman JW, Melo JV, Baccarani M, Thiele J. Chronic myelogenous leukaemia, BCR-ABL1 positive. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H. et al.editors WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC: Lyon: IARC; 2008. p. 32-7
- 4 Mishra P, Seth T, Mahapatra M, Saxena R. Report of chronic myeloid leukemia in chronic phase from all India Institute of Medical Sciences, 1990-2010. Indian J Med Paediatr Oncol 2013; 34: 159-63
- 5 Marin T, Butturini A, Kantarjian H, Sokal J, Mickey MR, Gale R. et al. Survival of children with chronic myeloid leukemia. Am J Pediatr Hematol Oncol 1992; 14: 229-32
- 6 Savage DG, Szydlo RM, Goldman JM. Clinical features at diagnosis in 430 patients with chronic myeloid leukaemia seen at a referral centre over a 16-year period. Br J Haematol 1997; 96: 111-6
- 7 Jakab Z, Balogh E, Kiss C, Oláh E. et al. Epidemiologic studies in a population-based childhood cancer registry in Northeast Hungary. Med Pediatr Oncol 2002; 38: 338-44
- 8 Millot F, Traore P, Guilhot J, Nelken B, Leblanc T, Leverger G. et al. Clinical and biological features at diagnosis in 40 children with chronic myeloid leukemia. Pediatrics 2005; 116: 140-3
- 9 Raut L, Bohara VV, Ray SS, Chakrabarti P, Chaudhuri U. Chronic myeloid leukemia in children and adolescents: A single center experience from Eastern India. South Asian J Cancer 2013; 2: 260-4
- 10 Smith KL, Johnson W. Classification of chronic myelocytic leukemia in children. Cancer 1974; 34: 670-9
- 11 Mendizabal AM, Garcia-Gonzalez P, Levine PH. Regional variations in age at diagnosis and overall survival among patients with chronic myeloid leukemia from low and middle income countries. Cancer Epidemiol 2013; 37: 247-54
- 12 Horibe K, Tsukimoto I, Ohno R. Clinicopathologic characteristics of leukemia in Japanese children and young adults. Leukemia 2001; 15: 1256-61
- 13 Cardamone JM, Edson JR, McArthur JR, Jacob HS. Abnormalities of platelet function in the myeloproliferative disorders. JAMA 1972; 221: 270-3
- 14 Hasan SK, Sazawal S, Kumar B, Chaubey R, Mishra P, Mir R. et al. Childhood CML in India: B2a2 transcript is more common than b3a2. Cancer Genet Cytogenet 2006; 169: 76-7
- 15 Jain P, Kantarjian H, Patel KP, Gonzalez GN, Luthra R, Kanagal ShamannaR. et al Impact of BCR-ABL transcript type on outcome in patients with chronic-phase CML treated with tyrosine kinase inhibitors. Blood 2016; 127: 1269-75
- 16 Hanfstein B, Lauseker M, Hehlmann R, Saussele S, Erben P, Dietz C. et al. Distinct characteristics of e13a2 versus e14a2 BCR-ABL1 driven chronic myeloid leukemia under first-line therapy with Imatinib. Haematologica 2014; 99: 1441-7
- 17 Rowe JM, Lichtman MA. Hyperleukocytosis and leukostasis: Common features of childhood chronic myelogenous leukemia. Blood 1984; 63: 1230-4