Philadelphia-positive atypical chronic myeloid leukemia, BCR/ABL negative: a case report


  • Germán Rivera Monroy Universidad Anáhuac México, Centro de Investigación en Ciencias de la Salud (CICSA), Facultad de Ciencias de la Salud
  • Federica Riccio Cuomo Universidad Anáhuac Puebla, Escuela de Medicina
  • Dunia Michelle Bustos Campa Universidad Anáhuac Puebla, Escuela de Medicina
  • Jorge Luis López Marthen Centro Médico Nacional Manuel Ávila Camacho (IMSS)


Palabras clave:

LMC atípica, Ph positivo, ABL/BCR negativo, hidroxiurea, imatinib


Atypical myeloid leukemia is a myeloproliferative neoplasm in which patients have a dysplastic increase in blood cells of the myeloid lineage as a result of hypercellular bone marrow. Our patient presented a positive Ph karyotype and a negative ABL/BCR transcript. Moreover, her age does not correspond to the mean of diagnosis. Her first treatment was hydroxyurea 1 g/kg for 60 days, which was not positive for the evolution of the disease and thus the elective treatment was changed to imatinib 400 mg/ day. After two months, the treatment has produced a favorable response, which leads to think that the therapy with imatinib offers better clinical results than other conventional ones. This text presents the study carried out at the Manuel Ávila Camacho National Medical Center and compares it with the existing literature regarding diagnosis and treatment to assess similarities and highlight specific points that may help in future case management.



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Lasho T. Atypical CML- the role of morphology and precision genomics. Best Pract Res Clin Haematol. 2020;33(2):101133.

Minciacchi VR, Kumar R, Krause DS. Chronic Myeloid Leukemia: A Model Disease of the Past, Present and Future. Cells, 2021; 10(1), 117.

Belkhair J, Raissi A, Elyahyaoui H, Ameur MA, Chakour M. Atypical chronic myeloid leukemia BCR-ABL 1 negative: A case report and literature review. Leuk Res Rep. 2019 25(12)100172

Onida F, Ball G, Kantarjian HM, Smith TL, Glassman A. Characteristics and outcome of patients with Philadelphia chromosome negative, bcr/abl negative chronic myelogenous leukemia. Cancer. 2002;95(8):1673–1684.

Jabbour E, Kantarjian H. Chronic myeloid leukemia: 2020 update on diagnosis, therapy and monitoring.Am J Hematol. 2020;95(6), 691–709.

Zhou T, Medeiros LJ, Hu S. Chronic Myeloid Leukemia: Beyond BCR-ABL1. Curr Hematol Malig Rep. 2018;13(6), 435–445.

Giri S, Pathak R, Martin MG, Bhatt VR. Characteristics and survival of BCR/ABL negative chronic myeloid leukemia: a retrospective analysis of the Surveillance, Epidemiology and End Results database. Ther Adv Hematol. 2015;6(6):308–12.

Sampaio MM, Santos M, Marques HS, Gonçalves V, Araújo G, Lopes LW, Apolonio JS, et al. Chronic myeloid leukemia-from the Philadelphia chromosome to specific target drugs: A literature review. World J Clin Oncol. 2021;12(2), 69–94.

Sadigh S, Hasserjian RP, Hobbs G. Distinguishing atypical chronic myeloid leukemia from other Philadelphia-negative chronic myeloproliferative neoplasms. Curr Opin Hematol. 2020;27(2), 122–127.

Ali MA. Chronic Myeloid Leukemia in the Era of Tyrosine Kinase Inhibitors: An Evolving Paradigm of Molecularly Targeted Therapy. Mol Diagn Ther. 2016;20(4), 315–333.

Narasimha M, Khamkar V, Tilwani S. et al. Atypical activation of signaling downstream of inactivated Bcr-Abl mediates chemoresistance in chronic myeloid leukemia. J. Cell Commun. Signal. (2021).

Iacobucci I, Lonetti A, Messa F, Cilloni D, Arruga F, Ottaviani E, et al. Expression of spliced oncogenic Ikaros isoforms in Philadelphia-positive acute lymphoblastic leukemia patients treated with tyrosine kinase inhibitors: implications for a new mechanism of resistance. Blood rev. 2009;112:3847–3855.

Schwartz LC, Mascarenhas J. Current and evolving understanding of atypical chronic myeloid leukemia. Blood rev. 2019;33, 74–81.

Quentmeier H, Eberth S, Romani J, Zaborski M, & Drexler HG.. (2011). BCR-ABL1-independent PI3Kinase activation causing imatinib-resistance. J hematology, 4, 6.

Issa GC, Kantarjian HM, Gonzalez GN, Borthakur G, Tang G, Wierda W, Sasaki K, et al. Clonal chromosomal abnormalities appearing in Philadelphia chromosome-negative metaphases during CML treatment. Blood rev. 2017;130(19), 2084–2091.





Cómo citar

Rivera Monroy, G. ., Riccio Cuomo, F., Bustos Campa, D. M. ., & López Marthen, J. L. . (2022). Philadelphia-positive atypical chronic myeloid leukemia, BCR/ABL negative: a case report. Proceedings of Scientific Research Universidad Anáhuac. Multidisciplinary Journal of Healthcare, 2(3), 36–40.



Case report

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