Mixed-Lineage Leukemia

Suci Iriani, Agus Alim Abdullah, Darwati Muhadi, Mansyur Arif

Abstract


Introduction: Acute Leukemia with Mixed Lineage phenotype (MLL) is leukemia that consists of cells characterized by mixed lineage markers, both from myeloid and lymphoid cells. The incidence of this leukemia is only 2-5% of all acute leukemias and is considered to have a poor prognosis.

Case Presentation: A seven-year-old girl was diagnosed with MLL. The results of immunophenotyping showed two blast populations with the expressions of CD33, CD34, HLA-DR, CD117, CD13, CD19, CD10, CD20, CyMPO, Cy CD79a, and morphological features of Acute Lymphoblastic Leukemia (ALL) and Acute nonLymphocytic Leukemia (AnLL) on bone marrow aspiration. The BCR-ABL examination showed the detected BCR-ABL p210 (Major breakpoint), the majority of which was found in chronic myeloid leukemia (CML) patients. There is no definite pathogenesis of BCR-ABL p210 (MBCR-ABL) in this patient. BCR-ABL can also present in 11–29% of ALL patients but is relatively rare in childhood ALL (1%–3%) and mostly expresses p190 (minor breakpoint (mBCR-ABL)). The p210 BCR-ABL transcript is detected in 30% of adults and 20% of childhood ALL patients with Philadelphia ALL.

Conclusions: MLL with BCR-ABL p210 transcript is very rare in acute leukemia. Immunophenotyping tests can detect typical MLL profiles, and WHO has standardized the diagnosis for MLL.


Keywords


acute lymphoblastic leukemia, BCR-ABL, mixed lineage leukemia

References


Yong WL, Yusof N, Ithnin A, et al. Mixed phenotype acute leukaemia with t(9,22), BCR-ABL1: A case report. Malays J Pathol. 2020;42(3):469-76.

Weinberg OK, Arber DA. Mixed-phenotype acute leukemia: historical overview and a new definition. Leukemia. 2010;24(11):1844-51.

Cernan M, Szotkowski T, Pikalova Z. Mixedphenotype acute leukemia: state-of-the-art of the diagnosis, classification and treatment. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2017;161(3):234-41.

Borowitz MJ. Acute leukaemias of ambiguous lineage. In: Swerdlow SH, Campo E, Harris NL, editors. WHO Classification of Tumours of Haematopoietic and the Lymphoid Tissues. 4th ed. Lyon: IARC Press; 2008. P. 150-155.

Weinberg OK, Seetharam M, Ren L, et al. Mixed phenotype acute leukemia: A study of 61 cases using World Health Organization and European Group for the Immunological Classification of Leukemias criteria. Am J Clin Pathol. 2014;142(6):803-8.

Lee GH, Baek HJ, Kim HS, et al. Biphenotypic acute leukemia or acute leukemia of ambiguous lineagein childhood: clinical characteristics and outcome. Blood Res. 2019;54:63-73.

Bene MC, Porwit A. Acute leukemias of ambiguous lineage. Semin Diagn Pathol. 2012;29:12-8.

Charles NJ, Boyer DF. Mixed-Phenotype Acute Leukemia: Diagnosis Criteria and Pittfalls. Arch Pathol Lab Med. 2017;141:1462-8.

Gerr H, Zimmermann M, Schrappe M, et al. Acute leukaemias of ambiguous lineage in children: characterization, prognosis and therapy recommendations. Br J Haematol. 2010;149(1):84–92.

Rossi JG, Bernasconi AR, Alonso CN, et al. Lineage switch in childhood acute leukemia: an unusual event with poor outcome. Am J Hematol. 2012;87(9):890–7.

Sholikah TA. Fusion gene bcr-abl : from etiopathogenesis to the management of Chronic Myeloid Leukemia. JKKI. 2017;8(1):29-37.

Kang ZJ, Liu YF, Xu LZ, et al. The Philadelphia chromosome in leukemogenesis. Chin J Cancer. 2016.

Bhatia P, Binota J, Varma N, et al. A Study on the Expression of BCR-ABL Transcript in Mixed Phenotype Acute Leukemia (MPAL) Cases Using the Reverse Transcriptase Polymerase Reaction Assay (RT-PCR) and its Correlation with Hematological Remission Status Post Initial Induction Therapy. Mediterr J Hematol Infect Dis. 2012;4(1):e2012024.

Chang TY, Chen SH, Jaing TH, et al. Cytogenetic abberation in mixed phenotype acute leukemia in children: A single-center retrospective review. Pediatrics & Neonatology. 2021;62(1);21-25

Omman RA, Kini AR. Acute leukemias. In: Keohane EM, Otto CN, Walenga JM, editors. Rodak’s Hematology. 6th ed. Elsevier; 2020.

Hoffbrand AV, Steensma DP. Essential Haematology. 8th ed. Sussex: Willey & Blackwell; 2019.

Chang VT, Aviv H, Howard LM, Padberg F. Acute Myelogenous Leukemia Associated With Extreme Symptomatic Thrombocytosis and Chromosome 3q Translocation: Case Report and Review of Literature. American Journal of Hematology. 2002;72:20–26.

You EK, Cho SY, Yang JJ, et al. Novel Case of Extreme Thrombocytosis in Acute Myeloid Leukemia Associated With Isochromosome 17q and Copy Neutral Loss of Heterozygosity. Ann Lab Med. 2015;35:366-9.

Malkan UY, Gunes G, Isik A, et al. Rebound Thrombocytosis following Induction Chemotherapy Is an Independent Predictor of a Good Prognosis in Acute Myeloid Leukemia Patients Attaining First Complete Remission. Acta Haematol. 2015;134:32–37.

Alexander TB, Gu Z, Iacobucci I, et al. The genetic basis and cell of origin of mixed phenotype acute leukaemia. Nature. 2018; 562(7727): 373-9.

Nishiwaki S, Kim JH, Ito M, et al. Multi-Lineage BCR-ABL Expression in Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia Is Associated With Improved Prognosis but No Specific Molecular Features. Front Oncol. 2020; 10:586567.

Kim HY, Hur M, Kim H, et al. First Case of Biphenotypic/bilineal (B/myeloid, B/monocytic) Mixed Phenotype Acute Leukemia with t(9;22)(q34;q11.2);BCR-ABL1. Annals of Clinical & Laboratory Scienc. 2016;46(4):435-8.


Full Text: PDF

DOI: 10.33371/ijoc.v16i3.875

Article Metrics

Abstract view : 65 times
PDF - 37 times

Refbacks

  • There are currently no refbacks.


Copyright (c) 2022 Indonesian Journal of Cancer

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.