Challenges and Opportunities in Management of Adult Mixed Phenotype Acute Leukaemia | Chapter 4 | Medical Research and Its Applications Vol. 6

Background & Objectives: Mixed-phenotype leukemia (MPAL) is a type of acute leukemia in which the blast population shows mixed features of myeloid, T-lymphoid, and/or B-lymphoid differentiation. MPALs are rare and carry a poor prognosis because of both diagnostic and therapeutic challenge. Conventionally, the diagnosis of MPAL requires either a single blast population with a lineage-defining phenotypic expression of multiple lineages (myeloid, B-cell and/or T-cell) (biphenotypic) or two distinct blast populations that each independently satisfy criteria for designation as AML, B-ALL, and/or T-ALL (bilineage). This retrospective study was designed to study the clinicopathological presentation and treatment outcome of MPAL cases in a tertiary care hospital in western India.

Aim: To evaluate the clinicopathological characteristics and treatment outcomes of patients with MPAL in a developing Low–middle–income country.

Methods: A retrospective study of 24 adult patients newly diagnosed with MPAL from January 2005 to December 2017.

Results: There were 8 females and 16 males. The average age was 35 years old (range = 19–78). Nine cases were bilineage and fifteen were biphenotypic. There were 21 adult and 3 geriatric patients.  We found 8 T/Myeloid (33.3%) and 16 B/Myeloid (66.7%) patients. MLL gene rearrangement was detected in Two (8.3%) patients. Philadelphia-positive chromosomes were detected in four (16.7%) patients, and FMS-like tyrosine kinase 3 (FLT3-ITD) internal tandem duplication (FLT3-ITD) was detected in two (8.3%) patients. CNS involvement was found at presentation in 3 (12.5%) cases among 24 MPAL patients.  MPAL patients were treated with either ALL or AML protocol combined with tyrosine kinase inhibitor (TKI). Eighteen patients (75%) received acute lymphoblastic leukemia (ALL) induction and thirteen patients (72.2%) achieved complete remission (CR) with no induction deaths. Therapy for four Ph1+ MPALs is based on an ALL or AML protocol combined with tyrosine kinase inhibitor (TKI). Six patients (25%) started therapy with acute myeloid leukemia (AML) induction: 3(50%) achieved CR. Five patients with ALL induction failure were switched to AML therapy. 5-year EFS 79.2% overall, 83.3% with ALL directed treatment and 66.7% with AML directed treatment. Five patients relapsed, three after ALL-directed treatment and two after AML-directed treatment.

Conclusion: ALL-directed therapy is associated with better outcomes than AML directed therapy in adult MPAL patients in our study.

 

Author(s)details:-

 

Shah Kajal
Department of Medical Oncology and Paediatrics Oncology, Gujarat Cancer Research Institute, India.

 

Panchal Harsha
Department of Medical Oncology and Paediatrics Oncology, Gujarat Cancer Research Institute, India.

 

Patel Apurva
Department of Medical Oncology and Paediatrics Oncology, Gujarat Cancer Research Institute, India.

 

Chinmay Doctor
Department of Medical Oncology and Paediatrics Oncology, Gujarat Cancer Research Institute, India.

 

Yadav Rajan
Department of Medical Oncology and Paediatrics Oncology, Gujarat Cancer Research Institute, India.

 

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