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Mixed-phenotype acute leukemia: state-of-the-art of the diagnosis, classification and treatment
Martin Cernan1, Tomas Szotkowski1, Zuzana Pikalova1
1Department of Hemato-Oncology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
Abstract:
Mixed-phenotype acute leukemia (MPAL) is a heterogeneous group of hematopoietic malignancies in which blasts show markers of multiple developmental lineages and cannot be clearly classified as acute myeloid or lymphoblastic leukemias. Historically, various names and classifications were used for this rare entity accounting for 2-5% of all acute leukemias depending on the diagnostic criterias used. The currently valid classification of myeloid neoplasms and acute leukemia published by the World Health Organization (WHO) in 2016 refers to this group of diseases as MPAL. Because adverse cytogenetic abnormalities are frequently present, MPAL is generally considered a disease with a poor prognosis. Knowledge of its treatment is limited to retrospective analyses of small patient cohorts. So far, no treatment recommendations verified by prospective studies have been published. The reported data suggest that induction therapy for acute lymphoblastic leukemia followed by allogeneic hematopoietic cell transplantation is more effective than induction therapy for acute myeloid leukemia or consolidation chemotherapy. The establishment of cooperative groups and international registries based on the recent WHO criterias are required to ensure further progress in understanding and treatment of MPAL. This review summarizes current knowledge on the diagnosis, classification, prognosis and treatment of MPAL patients.
Insights
Mixed-phenotype acute leukemia (MPAL) is a rare blood cancer. Current data suggest lymphoblastic leukemia induction therapy followed by stem cell transplant offers better outcomes for MPAL patients.
Area of Science:
- Hematology
- Oncology
- Molecular Biology
Background:
- Mixed-phenotype acute leukemia (MPAL) is a rare and heterogeneous hematopoietic malignancy.
- MPAL blasts exhibit markers from multiple cell lineages, defying clear myeloid or lymphoid classification.
- Historically, MPAL has been known by various names, representing 2-5% of acute leukemias.
Purpose of the Study:
- To review current knowledge on the diagnosis, classification, prognosis, and treatment of MPAL.
- To highlight the challenges in MPAL treatment due to its rarity and heterogeneity.
- To emphasize the need for cooperative groups and international registries for MPAL research.
Main Methods:
- Review of existing literature on MPAL diagnosis and classification based on WHO 2016 criteria.
- Analysis of retrospective data from small patient cohorts regarding treatment outcomes.
- Synthesis of current understanding of MPAL prognosis and therapeutic strategies.
Main Results:
- MPAL is characterized by frequent adverse cytogenetic abnormalities, indicating a poor prognosis.
- Limited treatment knowledge exists, primarily from retrospective analyses.
- Acute lymphoblastic leukemia induction therapy followed by allogeneic hematopoietic cell transplantation appears more effective than AML induction or consolidation chemotherapy.
Conclusions:
- MPAL is a challenging malignancy with a generally poor prognosis.
- Prospective studies are lacking, and treatment recommendations are not yet established.
- International collaboration and registries are crucial for advancing MPAL understanding and treatment.