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Acute Myeloid Leukemia with RAM Immunophenotype

MedGen UID:
1714533
•Concept ID:
C5238255
•
Neoplastic Process
Synonym: AML with RAM Immunophenotype

Definition

A high risk pediatric acute myeloid leukemia with an extremely poor prognosis. The blasts show bright CD56 expression, dim-to-negative expression of CD45 and CD38, and lack of HLA-DR expression. This immunophenotype was named after one of the pediatric patient''s initials (RAM), from Children''s Oncology Group (COG) clinical trial AAML0531. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute Myeloid Leukemia with RAM Immunophenotype

Recent clinical studies

Etiology

Immunophenotypic clustering in paediatric acute myeloid leukaemia.
Liu H, Wu K, Hu W, Chen X, Tang Y, Ma Y, Chen C, Xie Y, Yu L, Huang J, Shen S, Wang X
Br J Haematol 2024 Jun;204(6):2275-2286. Epub 2024 Apr 19 doi: 10.1111/bjh.19471. PMID: 38639201
Acute myeloid leukemia with RAM immunophenotype: A new underdiagnosed entity.
Gajendra S, Anupurba S, Gupta R, Mallick S, Panda D, Thakral D, Gupta SK, Bakhshi S, Seth R, Rai S, Prajapati VK, Singh S
Int J Lab Hematol 2023 Aug;45(4):541-552. Epub 2023 Apr 20 doi: 10.1111/ijlh.14076. PMID: 37078511
Utility of CD36 as a novel addition to the immunophenotypic signature of RAM-phenotype acute myeloid leukemia and study of its clinicopathological characteristics.
Panda D, Chatterjee G, Sardana R, Khanka T, Ghogale S, Deshpande N, Badrinath Y, Shetty D, Narula G, Banavali S, Patkar NV, Gujral S, Subramanian PG, Tembhare PR
Cytometry B Clin Cytom 2021 Mar;100(2):206-217. Epub 2020 Aug 31 doi: 10.1002/cyto.b.21943. PMID: 32865882
Deciphering the Significance of CD56 Expression in Pediatric Acute Myeloid Leukemia: A Report from the Children's Oncology Group.
Pardo LM, Voigt AP, Alonzo TA, Wilson ER, Gerbing RB, Paine DJ, Dai F, Menssen AJ, Raimondi SC, Hirsch BA, Gamis AS, Meshinchi S, Wells DA, Brodersen LE, Loken MR
Cytometry B Clin Cytom 2020 Jan;98(1):52-56. Epub 2019 Jul 11 doi: 10.1002/cyto.b.21829. PMID: 31294507Free PMC Article
Monozygotic twins diagnosed simultaneously with RAM immunophenotype acute myeloid leukemia.
Abu-Arja RF, Dargart JL, Bajwa RPS, Kahwash SB, Auletta JJ, Rangarajan HG
Pediatr Transplant 2018 Dec;22(8):e13291. Epub 2018 Sep 15 doi: 10.1111/petr.13291. PMID: 30220110

Diagnosis

Immunophenotypic clustering in paediatric acute myeloid leukaemia.
Liu H, Wu K, Hu W, Chen X, Tang Y, Ma Y, Chen C, Xie Y, Yu L, Huang J, Shen S, Wang X
Br J Haematol 2024 Jun;204(6):2275-2286. Epub 2024 Apr 19 doi: 10.1111/bjh.19471. PMID: 38639201
Acute myeloid leukemia with RAM immunophenotype: A new underdiagnosed entity.
Gajendra S, Anupurba S, Gupta R, Mallick S, Panda D, Thakral D, Gupta SK, Bakhshi S, Seth R, Rai S, Prajapati VK, Singh S
Int J Lab Hematol 2023 Aug;45(4):541-552. Epub 2023 Apr 20 doi: 10.1111/ijlh.14076. PMID: 37078511
Utility of CD36 as a novel addition to the immunophenotypic signature of RAM-phenotype acute myeloid leukemia and study of its clinicopathological characteristics.
Panda D, Chatterjee G, Sardana R, Khanka T, Ghogale S, Deshpande N, Badrinath Y, Shetty D, Narula G, Banavali S, Patkar NV, Gujral S, Subramanian PG, Tembhare PR
Cytometry B Clin Cytom 2021 Mar;100(2):206-217. Epub 2020 Aug 31 doi: 10.1002/cyto.b.21943. PMID: 32865882
Monozygotic twins diagnosed simultaneously with RAM immunophenotype acute myeloid leukemia.
Abu-Arja RF, Dargart JL, Bajwa RPS, Kahwash SB, Auletta JJ, Rangarajan HG
Pediatr Transplant 2018 Dec;22(8):e13291. Epub 2018 Sep 15 doi: 10.1111/petr.13291. PMID: 30220110
Childhood acute erythroleukemia diagnosis by flow cytometry.
Sharma A, Buxi G, Walia R, Yadav RB, Sharma S
Indian J Pathol Microbiol 2011 Jan-Mar;54(1):173-5. doi: 10.4103/0377-4929.77395. PMID: 21393910

Therapy

Deciphering the Significance of CD56 Expression in Pediatric Acute Myeloid Leukemia: A Report from the Children's Oncology Group.
Pardo LM, Voigt AP, Alonzo TA, Wilson ER, Gerbing RB, Paine DJ, Dai F, Menssen AJ, Raimondi SC, Hirsch BA, Gamis AS, Meshinchi S, Wells DA, Brodersen LE, Loken MR
Cytometry B Clin Cytom 2020 Jan;98(1):52-56. Epub 2019 Jul 11 doi: 10.1002/cyto.b.21829. PMID: 31294507Free PMC Article

Prognosis

Immunophenotypic clustering in paediatric acute myeloid leukaemia.
Liu H, Wu K, Hu W, Chen X, Tang Y, Ma Y, Chen C, Xie Y, Yu L, Huang J, Shen S, Wang X
Br J Haematol 2024 Jun;204(6):2275-2286. Epub 2024 Apr 19 doi: 10.1111/bjh.19471. PMID: 38639201
Acute myeloid leukemia with RAM immunophenotype: A new underdiagnosed entity.
Gajendra S, Anupurba S, Gupta R, Mallick S, Panda D, Thakral D, Gupta SK, Bakhshi S, Seth R, Rai S, Prajapati VK, Singh S
Int J Lab Hematol 2023 Aug;45(4):541-552. Epub 2023 Apr 20 doi: 10.1111/ijlh.14076. PMID: 37078511
Deciphering the Significance of CD56 Expression in Pediatric Acute Myeloid Leukemia: A Report from the Children's Oncology Group.
Pardo LM, Voigt AP, Alonzo TA, Wilson ER, Gerbing RB, Paine DJ, Dai F, Menssen AJ, Raimondi SC, Hirsch BA, Gamis AS, Meshinchi S, Wells DA, Brodersen LE, Loken MR
Cytometry B Clin Cytom 2020 Jan;98(1):52-56. Epub 2019 Jul 11 doi: 10.1002/cyto.b.21829. PMID: 31294507Free PMC Article
Monozygotic twins diagnosed simultaneously with RAM immunophenotype acute myeloid leukemia.
Abu-Arja RF, Dargart JL, Bajwa RPS, Kahwash SB, Auletta JJ, Rangarajan HG
Pediatr Transplant 2018 Dec;22(8):e13291. Epub 2018 Sep 15 doi: 10.1111/petr.13291. PMID: 30220110

Clinical prediction guides

Immunophenotypic clustering in paediatric acute myeloid leukaemia.
Liu H, Wu K, Hu W, Chen X, Tang Y, Ma Y, Chen C, Xie Y, Yu L, Huang J, Shen S, Wang X
Br J Haematol 2024 Jun;204(6):2275-2286. Epub 2024 Apr 19 doi: 10.1111/bjh.19471. PMID: 38639201
Acute myeloid leukemia with RAM immunophenotype: A new underdiagnosed entity.
Gajendra S, Anupurba S, Gupta R, Mallick S, Panda D, Thakral D, Gupta SK, Bakhshi S, Seth R, Rai S, Prajapati VK, Singh S
Int J Lab Hematol 2023 Aug;45(4):541-552. Epub 2023 Apr 20 doi: 10.1111/ijlh.14076. PMID: 37078511
Role of CD71 in acute leukemia- An immunophenotypic marker for erythroid lineage or proliferation?
Acharya S, Kala PS
Indian J Pathol Microbiol 2019 Jul-Sep;62(3):418-422. doi: 10.4103/IJPM.IJPM_604_18. PMID: 31361230

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