Mixed phenotype acute leukemia: Biological profile, clinical characteristic and treatment outcomes: Report of the population-based study

Eur J Haematol. 2020 Jul;105(1):85-93. doi: 10.1111/ejh.13413. Epub 2020 Apr 17.

Abstract

Objectives: The aim of this population-based, retrospective study was to analyze biological and clinical features and treatment results in children diagnosed with MPAL in all Polish pediatric oncology centers between 2007 and 2018.

Methods: Among 2893 children and adolescents diagnosed and treated for acute leukemia, 39 (1.35%) patients fulfilled the WHO criteria of MPAL. The T/myeloid phenotype was most prevalent.

Results: Cytogenetics findings were seen in 2 (5.1%), while chromosomal abnormalities were found in 14 (35.9%) patients. Thirty-two patients achieved CR-1, including 23 (92.0%) treated with ALL-directed chemotherapy and 9 (64.3%) treated with AML-type induction regimens. Within these patients, 4 (12.5%) died due to treatment-related complications and 11 (34.4%) relapsed. Nineteen (63.3%) patients underwent allo-HSCT in CR-1 and 14 (73.7%) of them have been in CR-1. In total, 17 (43.6%) patients remain in CR-1 for 1-12 years, including 14 (58.3%) with T/myeloid MPAL. The 5-year pOS and pEFS were 51.8% and 44.2%, respectively. The overall survival for ALL-directed therapy was significantly better than the one for AML-type chemotherapy (P = .001). It was also better for patients who underwent HSCT in CR-1 (P = .001).

Conclusions: The prognosis of MPAL is unsatisfactory, but initial treatment with ALL-directed chemotherapy consolidated with allo-HSCT improves the outcomes in MPAL.

Keywords: chemotherapy; diagnosis; hematopoietic stem cell transplantation; mixed phenotype acute leukemia; prognosis.

MeSH terms

  • Clinical Decision-Making
  • Combined Modality Therapy / adverse effects
  • Combined Modality Therapy / methods
  • Disease Management
  • Disease Susceptibility
  • Genetic Predisposition to Disease
  • Humans
  • Leukemia, Biphenotypic, Acute / diagnosis
  • Leukemia, Biphenotypic, Acute / epidemiology*
  • Leukemia, Biphenotypic, Acute / etiology
  • Leukemia, Biphenotypic, Acute / therapy
  • Phenotype
  • Poland / epidemiology
  • Public Health Surveillance
  • Retrospective Studies
  • Treatment Outcome