High-Risk Acute Promyelocytic Leukemia with Unusual T/Myeloid Immunophenotype Successfully Treated with ATRA and Arsenic Trioxide-Based Regimen.
Zeba N SinghVu H DuongRima KokaYing ZouSameer SawhneyLi TangMaria R BaerNicholas AmbulosFiras El ChaerAshkan EmadiPublished in: Journal of hematopathology (2018)
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.
Keyphrases
- acute myeloid leukemia
- stem cell transplantation
- hematopoietic stem cell
- high dose
- bone marrow
- end stage renal disease
- single cell
- drinking water
- ejection fraction
- newly diagnosed
- poor prognosis
- heavy metals
- chronic kidney disease
- prognostic factors
- liver failure
- high frequency
- gene expression
- systemic lupus erythematosus
- mesenchymal stem cells
- immune response
- squamous cell carcinoma
- low dose
- intensive care unit
- disease activity
- locally advanced
- smoking cessation
- tyrosine kinase
- long non coding rna
- rheumatoid arthritis
- replacement therapy
- data analysis