Abstract
Mixed phenotype acute leukemia (MPAL) is a rare type of leukemia with a limited number of studies conducted to characterize its clinical spectrum and most importantly the best treatment modality. MPAL blasts show more than one phenotype either myeloid/monocytic with T- or B-lymphoid or extremely rare triple lineage associated phenotypic markers. This study aimed to characterize MPAL cases with special emphasis on comparing adult and pediatric age groups, exploring treatment regimens, and clinical outcome. Among 2571 acute leukemia patients, 102 MPAL cases fulfilling the 2008/2016 WHO diagnostic criteria of MPAL were recruited in the study. The incidence of MPAL was 4% of acute leukemia patients. Pediatric cases were 54 (53%) while adults were 48/102 (47%). Myeloid/B-lymphoid phenotype was found in 86/102 (84%), with BCR-ABL fusion gene transcript detected in 14/102(13.7%) patients. ALL-like treatment showed better response rates as compared with the myeloid based regimen (p = 0.001). MPAL behaves in a manner that resembles in clinical features, their lymphoid progenitor counterpart leukemias both in adults and pediatric patients with superior treatment response to ALL-like regimen, especially in adults.
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ESM 1
Figure (1a): Dot plots of a Myeloid /T case; R1: Gating on CD45 dim population (blasts), R2: co-expression of MPO/cytoplasmic CD3, R3: co-expression of cytoplasmic CD13/cytoplasmic CD3, R4: co-expression of CD33/cytoplasmic CD3 (JPG 353 kb)
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Figure (1b): Dot plots of a Myeloid /B case; R1: Gating on CD45 dim population (blasts), R2: co-expression of CD19/ CD33, R3: co-expression of MPO/CD19 (JPG 664 kb)
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Figure (1c): 3D plot for cCD3 PE (Z axis) / CD33 PC5.5 (Y axis) / CD19 PC7 (X axis) expression in a case with T/B/Myeloid antigens expression (JPG 37 kb)
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Figure (1d): Dot plots for using residual normal lymphocytes as an internal negative control for MPO; R1: CD45 bright lymphocytes, R2: CD45 dim blasts, R3: negative MPO expression in lymphocytes, R4: MPO expression in blasts population. (JPG 379 kb)
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Rasekh, E.O., Osman, R., Ibraheem, D. et al. Acute lymphoblastic leukemia–like treatment regimen provides better response in mixed phenotype acute leukemia: a comparative study between adults and pediatric MPAL patients. Ann Hematol 100, 699–707 (2021). https://doi.org/10.1007/s00277-020-04354-2
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DOI: https://doi.org/10.1007/s00277-020-04354-2