Article Sidebar
Main Article Content
Abstract
Latar Belakang : Chronic myeloid leukemia (CML) sebagian besar ditemukan pada kelompok usia
dewasa dengan rata-rata usia 65 tahun. Meskipun jarang, CML juga dapat terjadi pada usia anak-anak
dengan angka insiden rata - rata sebesar 2,5 kasus per juta orang pertahun di dunia. Di Indonesia
sendiri, kasus CML anak mencapai 2,7% dari total kasus kanker pada tahun 2020-2024.
Kasus : Kami melaporkan seorang anak perempuan berusia 15 tahun yang datang dengan keluhan
lemas sejak 3 hari sebelum dibawa ke rumah sakit dan sudah berulang 5 tahun terakhir ini. Keluhan
disertai rambut sering rontok, berat badan tidak naik, perut semakin membesar, kulit terlihat menghitam
setiap hari dan demam berulang tanpa sebab yang jelas. Dari alloanamnesis ditemukan paman pasien
mengalami keluhan serupa. Pemeriksaan fisik didapatkan kedua konjungtiva anemis dan splenomegali.
Pemeriksaan hematologi didapatkan anemia, leukositosis dengan sel blast 9% dan trombositosis. Hasil
pemeriksaan molekuler BCR ABL didapatkan positif dari fusi gen BCR ABL exon e14a2, sehingga
pasien di diagnosis sebagai chronic myeloid leukemia phase kronis. Pasien dirawat di rumah sakit serta
diberikan terapi cairan dan Imatinib.
Background : Chronic myeloid leukemia (CML) is primarily found in adults, with an average age of 65
years. Although rare, it can also occur in children, with an average incidence of 2.5 cases per million
people per year worldwide. In Indonesia, 2.7% of total cancer cases between 2020 and 2024 was
pediatric CML.
Case : We report a 15-year-old girl who came to hospital with complaints of weakness for three days
prior to hospitalization, which had recurred over the past five years. Associated symptoms included
frequent hair loss, no weight gain, abdominal distension, daily skin darkening, and recurrent fever
without a clear cause. Family history revealed similar complaints were found in the patient's uncle.
Physical examination showed conjunctival pallor and splenomegaly. Anemia, leukocytosis with 9% blast
cells, and thrombocytosis were found in hematological test. Molecular testing for BCR ABL fusion gene
was positive for BCR ABL exon e14a2, leading to a diagnosis of chronic myeloid leukemia at chronic
phase. The patient was hospitalized and was given fluid therapy and Imatinib
Keywords
Article Details
Copyright (c) 2024 Mulyadi, Renillia, Dessy
This work is licensed under a Creative Commons Attribution-ShareAlike 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).
References
- Khalid, R., & Riasat, S. (2023). Molecular Pathogenesis and Treatment Strategies of Chronic Myeloid Leukemia (CML). Sudan Journal of Medical Sciences, 18(4), 525–538. https://doi.org/10.18502/sjms.v18i4.14741
- Minciacchi, V., Kumar, R., & Krause, D. (2021). Chronic myeloid leukemia: a model disease of the past, present and future. Cells, 10, 117. https://doi.org/10.1007/BF01532014
- Suttorp, M., Millot, F., Sembill, S., Deutsch, H., & Metzler, M. (2021). Definition, epidemiology, pathophysiology, and essential criteria for diagnosis of pediatric chronic myeloid leukemia. Cancers, 13(4), 1–21. https://doi.org/10.3390/cancers13040798
- Kementerian Kesehatan Republik Indonesia. (2023). Pedoman Penemuan Dini Kanker pada Anak.
- Indonesian Pediatric Cancer Registry. (2024). Mengungkap tantangan dan peluang dalam perawatan kanker anak di Indonesia: Data IPCAR 2020-2024.
- Rinaldi, I., & Winston, K. (2023). Chronic Myeloid Leukemia, from Pathophysiology to TreatmentFree Remission: A Narrative Literature Review. Journal of Blood Medicine, 14(April), 261–277. https://doi.org/10.2147/JBM.S382090
- Osman, A. E. G., & Deininger, M. W. (2021). Chronic Myeloid Leukemia: Modern therapies, current challenges and future directions. Blood Reviews, 49, 1–36. https://doi.org/10.1016/j.blre.2021.100825
- Jabbour, E., & Kantarjian, H. (2022). Chronic myeloid leukemia: 2022 update on diagnosis, therapy, and monitoring. American Journal of Hematology, 97(9), 1236–1256. https://doi.org/10.1002/ajh.26642
- Cortes, J. E., Saglio, G., Kantarjian, H. M., Baccarani, M., Mayer, J., Boqué, C., Shah, N. P., Chuah, C., Casanova, L., Bradley-Garelik, B., Manos, G., & Hochhaus, A. (2016). Final 5-year study results of DASISION: The dasatinib versus imatinib study in treatment-Naïve chronic myeloid leukemia83 Jurnal Kedokteran Raflesia Vol. 10 (2) 2024 patients trial. Journal of Clinical Oncology, 34(20), 2333–2340. https://doi.org/10.1200/JCO.2015.64.8899
- Hochhaus, A., Saglio, G., Hughes, T. P., Larson, R. A., Kim, D. W., Issaragrisil, S., Le Coutre, P. D., Etienne, G., Dorlhiac-Llacer, P. E., Clark, R. E., Flinn, I. W., Nakamae, H., Donohue, B., Deng, W., Dalal, D., Menssen, H. D., & Kantarjian, H. M. (2016). Long-term benefits and risks of frontline nilotinib vs imatinib for chronic myeloid leukemia in chronic phase: 5-year update of the randomized
- ENESTnd trial. Leukemia, 30(5), 1044–1054. https://doi.org/10.1038/leu.2016.5
- Bintoro, S. U. Y. (2019). Chronic Myeloid Leukimia. In Journal of Chemical Information and Modeling (Vol. 53, Issue 9).
- Urgessa, F., Lengiso, B., Tsegaye, A., Gebremedhin, A., Abdella, F., Tadesse, F., Radich, J., Nigussie, H., & Kuru Gerbaba, T. (2024). Hematological, clinical, cytogenetic and molecular profiles of confirmed chronic myeloid leukemia patients at presentation at a tertiary care teaching hospital in Addis Ababa, Ethiopia: a cross-sectional study. BMC Cancer, 24(1), 1–10. https://doi.org/10.1186/s12885-024-12282-x
- Vaidya, S. (2014). Imatinib resistant Chronic Myeloid Leukemia and therapeutic approach. ICMR, 45(1), 1–20.
- Eden, R., & Coviello, J. (2023). Chronic myelogenous leukemia. StatPearls.
- Iezza, M., Cortesi, S., Ottaviani, E., Mancini, M., Venturi, C., Monaldi, C., De Santis, S., Testoni, N., Soverini, S., Rosti, G., Cavo, M., & Castagnetti, F. (2023). Prognosis in Chronic Myeloid Leukemia: Baseline Factors, Dynamic Risk Assessment and Novel Insights. Cells, 12(13), 1–25. https://doi.org/10.3390/cells12131703
References
Khalid, R., & Riasat, S. (2023). Molecular Pathogenesis and Treatment Strategies of Chronic Myeloid Leukemia (CML). Sudan Journal of Medical Sciences, 18(4), 525–538. https://doi.org/10.18502/sjms.v18i4.14741
Minciacchi, V., Kumar, R., & Krause, D. (2021). Chronic myeloid leukemia: a model disease of the past, present and future. Cells, 10, 117. https://doi.org/10.1007/BF01532014
Suttorp, M., Millot, F., Sembill, S., Deutsch, H., & Metzler, M. (2021). Definition, epidemiology, pathophysiology, and essential criteria for diagnosis of pediatric chronic myeloid leukemia. Cancers, 13(4), 1–21. https://doi.org/10.3390/cancers13040798
Kementerian Kesehatan Republik Indonesia. (2023). Pedoman Penemuan Dini Kanker pada Anak.
Indonesian Pediatric Cancer Registry. (2024). Mengungkap tantangan dan peluang dalam perawatan kanker anak di Indonesia: Data IPCAR 2020-2024.
Rinaldi, I., & Winston, K. (2023). Chronic Myeloid Leukemia, from Pathophysiology to TreatmentFree Remission: A Narrative Literature Review. Journal of Blood Medicine, 14(April), 261–277. https://doi.org/10.2147/JBM.S382090
Osman, A. E. G., & Deininger, M. W. (2021). Chronic Myeloid Leukemia: Modern therapies, current challenges and future directions. Blood Reviews, 49, 1–36. https://doi.org/10.1016/j.blre.2021.100825
Jabbour, E., & Kantarjian, H. (2022). Chronic myeloid leukemia: 2022 update on diagnosis, therapy, and monitoring. American Journal of Hematology, 97(9), 1236–1256. https://doi.org/10.1002/ajh.26642
Cortes, J. E., Saglio, G., Kantarjian, H. M., Baccarani, M., Mayer, J., Boqué, C., Shah, N. P., Chuah, C., Casanova, L., Bradley-Garelik, B., Manos, G., & Hochhaus, A. (2016). Final 5-year study results of DASISION: The dasatinib versus imatinib study in treatment-Naïve chronic myeloid leukemia83 Jurnal Kedokteran Raflesia Vol. 10 (2) 2024 patients trial. Journal of Clinical Oncology, 34(20), 2333–2340. https://doi.org/10.1200/JCO.2015.64.8899
Hochhaus, A., Saglio, G., Hughes, T. P., Larson, R. A., Kim, D. W., Issaragrisil, S., Le Coutre, P. D., Etienne, G., Dorlhiac-Llacer, P. E., Clark, R. E., Flinn, I. W., Nakamae, H., Donohue, B., Deng, W., Dalal, D., Menssen, H. D., & Kantarjian, H. M. (2016). Long-term benefits and risks of frontline nilotinib vs imatinib for chronic myeloid leukemia in chronic phase: 5-year update of the randomized
ENESTnd trial. Leukemia, 30(5), 1044–1054. https://doi.org/10.1038/leu.2016.5
Bintoro, S. U. Y. (2019). Chronic Myeloid Leukimia. In Journal of Chemical Information and Modeling (Vol. 53, Issue 9).
Urgessa, F., Lengiso, B., Tsegaye, A., Gebremedhin, A., Abdella, F., Tadesse, F., Radich, J., Nigussie, H., & Kuru Gerbaba, T. (2024). Hematological, clinical, cytogenetic and molecular profiles of confirmed chronic myeloid leukemia patients at presentation at a tertiary care teaching hospital in Addis Ababa, Ethiopia: a cross-sectional study. BMC Cancer, 24(1), 1–10. https://doi.org/10.1186/s12885-024-12282-x
Vaidya, S. (2014). Imatinib resistant Chronic Myeloid Leukemia and therapeutic approach. ICMR, 45(1), 1–20.
Eden, R., & Coviello, J. (2023). Chronic myelogenous leukemia. StatPearls.
Iezza, M., Cortesi, S., Ottaviani, E., Mancini, M., Venturi, C., Monaldi, C., De Santis, S., Testoni, N., Soverini, S., Rosti, G., Cavo, M., & Castagnetti, F. (2023). Prognosis in Chronic Myeloid Leukemia: Baseline Factors, Dynamic Risk Assessment and Novel Insights. Cells, 12(13), 1–25. https://doi.org/10.3390/cells12131703