Acute lymphoblastic leukemia–like treatment regimen provides better response in mixed phenotype acute leukemia: a comparative study between adults and pediatric MPAL patients

When Flow Is Turbulent: CD19 Intensity Is a Pitfall in Acute Leukemia Classification

“…Retrospective data support preferred treatment of B/myeloid MPAL with a B-lymphoblastic leukemia chemotherapy regimen, rather than an AML regimen, 7–10 and recent reports including a series from our institution describe the use of blinatumomab in relapsed or refractory CD19 + MPAL with promising clinical response 11–15 . The phenomenon of lineage switch has been reported in the context of treatment of acute leukemias, 16 including recently in cases of MPAL and cases treated with blinatumomab, 15,17,18 although it is debatable whether the current case would constitute a lineage switch given the sequence of clinical events and lack of a true relapse 19 …”

Pediatric Mixed-Phenotype Acute Leukemia: What’s New?

“…Poor prognostic factors include: an older age at diagnosis, higher white blood cell (WBC) count at presentation, T-lymphoid/myeloid phenotype, adverse cytogenetics (such as a KMT2A / AFF1 rearrangement), extramedullary disease at diagnosis, and MRD positivity [ 11 , 33 , 34 ]. There have been various chemotherapy approaches for the treatment of MPAL including acute ALL, AML, and hybrid ALL/AML (such as FLAG (fludrabine, cytarabine, granulocyte-stimulating factor)-IDA (idarubicin) with vincristine and prednisone (VCR-PRED) or hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) regimens) [ 8 , 35 , 36 ]. Optimal therapy remains a subject of controversy and differences between adult and pediatric treatment approaches are often striking [ 8 , 37 ].…”

“…However, more recently, due to improved diagnostic criteria and genomic techniques, and the observation that ALL-like regimens in both pediatric and adult populations are associated with superior treatment response, the treatment landscape has clearly changed [ 5 , 35 , 39 , 40 , 41 ].…”

“…Additionally, a recent multi-center analysis showed that ALL induction therapy was able to achieve MRD negative remission by the end of induction in a majority of patients (70%) [ 40 ]. Even in studies showing similar survival benefits between ALL and AML/hybrid regimens, ALL regimens tend to lead to overall decreased morbidity, due to less toxicity, compared with AML or hybrid-based treatments [ 35 ]. Lumbar puncture is recommended at diagnosis to determine central nervous system (CNS) status for all MPAL patients, as frequency of CNS involvement is higher [ 7 , 45 ].…”

Paediatric ambiguous lineage leukaemia with monocytic differentiation at diagnosis: case series and review of literature

“…5,9 Previous studies have shown non-inferior outcomes in both CD19 + and CD19 À cases compared to non-ALAL cases with MRD 0Á1% or higher after induction treated on current intensified protocols. 5,12,13 We conducted a multicentre retrospective study with chart and pathology review of all cases of MPAL/ALAL diagnosed and treated at University Hospital Southampton (UHS) and Sheffield Children's Hospital (SCH), UK from the year 2015 until 2020. A total of three children with bilineal and biphenotypic acute leukaemia and a predominant monocytic component (UHS, one; SCH, two) were enrolled.…”