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Mixed Phenotype Acute Leukemia, T/Myeloid, Not Otherwise Specified

MedGen UID:
443132
•Concept ID:
C2826055
•
Neoplastic Process
Synonyms: Mixed phenotype acute leukemia T/myeloid; Mixed phenotype acute leukemia with myeloid and T-cell lymphoid phenotypes; Mixed Phenotype Acute Leukemia, T/Myeloid; Mixed Phenotype Acute Leukemia, T/Myeloid, NOS; MPAL, T/Myeloid, NOS
SNOMED CT: Mixed phenotype acute leukemia T/myeloid (450917002); Mixed phenotype acute leukemia with myeloid and T-cell lymphoid phenotypes (450917002)

Definition

A rare mixed phenotype acute leukemia in which the blasts express T-lymphoid and myeloid lineage markers but are negative for KMT2A rearrangement and t(9;22)(q34;q11.2) translocation. The prognosis is usually unfavorable. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Mixed Phenotype Acute Leukemia, T/Myeloid, Not Otherwise Specified

Professional guidelines

PubMed

Diagnosis and treatment of mixed phenotype (T-myeloid/lymphoid) acute leukemia with novel ETV6-FGFR2 rearrangement.
Carll T, Patel A, Derman B, Hyjek E, Lager A, Wanjari P, Segal J, Odenike O, Fidai S, Arber D
Blood Adv 2020 Oct 13;4(19):4924-4928. doi: 10.1182/bloodadvances.2019001282. PMID: 33049052Free PMC Article
Mixed phenotype acute leukemia: Biological profile, clinical characteristic and treatment outcomes: Report of the population-based study.
Zając-Spychała O, Irga-Jaworska N, Drożyńska E, Muszyńska-Rosłan K, Krawczuk-Rybak M, Zawitkowska J, Kowalczyk J, Ćwiklińska M, Balwierz W, Mizia-Malarz A, Badowska W, Kamieńska E, Urasiński T, Kaczorowska A, Kazanowska B, Chybicka A, Wysocki M, Sędek Ł, Szczepański T, Woszczyk M, Matysiak M, Młynarski W, Karolczyk G, Chaber R, Wachowiak J
Eur J Haematol 2020 Jul;105(1):85-93. Epub 2020 Apr 17 doi: 10.1111/ejh.13413. PMID: 32223036

Recent clinical studies

Etiology

14q32 rearrangements deregulating BCL11B mark a distinct subgroup of T-lymphoid and myeloid immature acute leukemia.
Di Giacomo D, La Starza R, Gorello P, Pellanera F, Kalender Atak Z, De Keersmaecker K, Pierini V, Harrison CJ, Arniani S, Moretti M, Testoni N, De Santis G, Roti G, Matteucci C, Bassan R, Vandenberghe P, Aerts S, Cools J, Bornhauser B, Bourquin JP, Piazza R, Mecucci C
Blood 2021 Sep 2;138(9):773-784. doi: 10.1182/blood.2020010510. PMID: 33876209Free PMC Article

Diagnosis

14q32 rearrangements deregulating BCL11B mark a distinct subgroup of T-lymphoid and myeloid immature acute leukemia.
Di Giacomo D, La Starza R, Gorello P, Pellanera F, Kalender Atak Z, De Keersmaecker K, Pierini V, Harrison CJ, Arniani S, Moretti M, Testoni N, De Santis G, Roti G, Matteucci C, Bassan R, Vandenberghe P, Aerts S, Cools J, Bornhauser B, Bourquin JP, Piazza R, Mecucci C
Blood 2021 Sep 2;138(9):773-784. doi: 10.1182/blood.2020010510. PMID: 33876209Free PMC Article
Pseudo-Chédiak-Higashi granules and Auer rods in mixed phenotype acute leukaemia, T/myeloid, not otherwise specified.
Aruga Y, Arakawa A, Ono K, Ogawa C, Matsushita H
Br J Haematol 2018 Jan;180(2):175. Epub 2017 Sep 29 doi: 10.1111/bjh.14924. PMID: 28961305

Therapy

14q32 rearrangements deregulating BCL11B mark a distinct subgroup of T-lymphoid and myeloid immature acute leukemia.
Di Giacomo D, La Starza R, Gorello P, Pellanera F, Kalender Atak Z, De Keersmaecker K, Pierini V, Harrison CJ, Arniani S, Moretti M, Testoni N, De Santis G, Roti G, Matteucci C, Bassan R, Vandenberghe P, Aerts S, Cools J, Bornhauser B, Bourquin JP, Piazza R, Mecucci C
Blood 2021 Sep 2;138(9):773-784. doi: 10.1182/blood.2020010510. PMID: 33876209Free PMC Article

Clinical prediction guides

ADAMTS2 gene dysregulation in T/myeloid mixed phenotype acute leukemia.
Tota G, Coccaro N, Zagaria A, Anelli L, Casieri P, Cellamare A, Minervini A, Minervini CF, Brunetti C, Impera L, Carluccio P, Cumbo C, Specchia G, Albano F
BMC Cancer 2014 Dec 16;14:963. doi: 10.1186/1471-2407-14-963. PMID: 25515027Free PMC Article

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