High-risk acute promyelocytic leukemia with unusual T/myeloid immunophenotype successfully treated with ATRA and arsenic trioxide-based regimen

Zeba N. Singh, Vu H. Duong, Rima Koka, Ying Zou, Sameer Sawhney, Li Tang, Maria R. Baer, Nicholas Ambulos, Firas El Chaer, Ashkan Emadi

Research output: Contribution to journalArticlepeer-review

Abstract

We describe two patients with acute promyelocytic leukemia (APL) with an unusual immunophenotype with co-expression of myeloperoxidase (MPO) with cytoplasmic CD3 (cCD3) representing myeloid and T-lineage differentiation. Both harbored FLT3-ITD mutations. One additionally had a deletion in the PML gene affecting the primer binding site, thus limiting measurable residual disease (MRD) analysis during follow-up. Both patients achieved durable remission with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO)-based therapy, thus mitigating the need for repetitive conventional chemotherapy cycles and allogeneic stem cell transplantation. Our report highlights the complexity and challenge of diagnosis and management of APL due to the variant immunophenotype and genetics and underscores the importance of synthesizing information from all testing modalities. The association of the unusual immunophenotype and FLT3-ITD mutation illustrates the plasticity of the hematopoietic stem cell and the pathobiology of leukemia with mixed lineage or lineage infidelity.

Original languageEnglish (US)
Pages (from-to)67-74
Number of pages8
JournalJournal of Hematopathology
Volume11
Issue number3
DOIs
StatePublished - Sep 1 2018
Externally publishedYes

Keywords

  • Acute promyelocytic leukemia (APL)
  • All-trans retinoic acid (ATRA)
  • Arsenic trioxide (ATO)
  • Measurable (minimal) residual disease (MRD)
  • Mixed phenotype acute leukemia (MPAL)

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Hematology

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