Abstract
Simple SummaryPediatric mixed-phenotype leukemia is a rare form of blood cancer in children. In this review, we cover both the evolution of treatment over the past several years and outline new emerging concepts in this disease. Mixed-phenotype acute leukemias (MPAL) are rare in children and often lack consensus on optimal management. This review examines the current controversies and emerging paradigms in the management of pediatric MPAL. We examine risk stratification, outcomes of recent retrospective and prospective collaborative trials, and the role of transplantation and precision genomics, and outline emerging targets and concepts in this rare entity.
Highlights
After Mixed-phenotype acute leukemias (MPAL) were recognized as a distinct entity, numerous sets of diagnostic criteria were established, including the European Group for the Immunological Characterization of Leukemias (EGIL), and most recently the World Health Organization (WHO) 2016 system, which is being increasingly utilized for MPAL diagnosis [6,9,10]
This is of particular interest with the increased use of B-ALL-directed immunotherapy, which has been linked to lineage switch, particuuse of B-ALL-directed immunotherapy, which has been linked to lineage switch, in KMT2A-rearranged leukemias [20,21,26,30,31]
MPAL are a heterogeneous group of leukemias that often have a complex phenotype/genetic basis and historically have been difficult to diagnose and treat
Summary
MPAL are heterogeneous and can exhibit cross-lineage myeloid, B-lymphoid, or T-lymphoid antigen expression on a single blast population (biphenotypic) or have distinct single-lineage blast populations (bilineal) [3]. Due to phenotypic and genetic diversity, lack of welldefined diagnostic criteria, treatment resistance, and lineage switch, MPAL often present a diagnostic dilemma, and prove difficult to treat. Biphenotypic MPAL are more common than bilineal MPAL. The true prevalence and survival can be difficult to determine given the various diagnostic criteria utilized, lack of a centralized review of cases, and treatment protocols that are based on results from retrospective studies. Lineage Task Force reported that routine institutional flow cytometry was insufficient for the diagnosis of MPAL in about 15% of children, which further highlights the diagnostic challenge faced by oncologists [5]
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