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Bilaga 4 Klassifikation av AML enligt ICC 2022

Hierarchical classification of the International Consensus Classification of AML [1]

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AML and related neoplasms and acute leukemias of ambiguous lineage [1]

AML and related neoplams

AML with recurrent genetic abnormalities (requiring ≥10% blasts in BM or PB)*

  • APL with t(15;17)(q24.1;q21.2)/PML::RARA†
  • AML with t(8;21)(q22;q22.1)/RUNX1::RUNX1T1
  • AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22)/CBFB::MYH11
  • AML with t(9;11)(p21.3;q23.3)/MLLT3::KMT2A‡
  • AML with t(6;9)(p22.3;q34.1)/DEK::NUP214
  • AML with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2)/GATA2, MECOM(EVI1)§
  • AML with other rare recurring translocations‖
  • AML with mutated NPM1
  • AML with in-frame bZIP mutated CEBPA¶
  • AML with t(9;22)(q34.1;q11.2)/BCR::ABL1* 

Myeloid sarcoma

Acute leukemia of ambiguous lineage

  • Acute undifferentiated leukemia
  • MPAL with t(9;22)(q34.1;q11.2)/BCR::ABL1
  • MPAL with t(v;11q23.3)/KMT2A-rearranged
  • MPAL, B/myeloid, not otherwise specified
  • MPAL, T/myeloid, not otherwise specified 

 

Categories designated AML (if ≥20% blasts in BM or PB) or MDS/AML (if 10-19% blasts in BM or PB)

  •  AML with mutated TP53#
  •  AML with myelodysplasia-related gene mutations
     Defined by mutations in ASXL1, BCOR, EZH2, RUNX1, SF3B1, SRSF2, STAG2, U2AF1, and/or ZRSR2
  •  AML with myelodysplasia-related cytogenetic abnormalities**
  • AML not otherwise specified
  •  

 

Myeloid proliferations related to Down syndrome

  • Transient abnormal myelopoiesis associated with Down syndrome
  • Myeloid leukemia associated with Down syndrome
Blastic plasmacytoid dendritic cell neoplasm

 

Diagnostic qualifiers††

Therapy-related‡‡

  • Prior chemotherapy, radiotherapy, immune interventions

Progressed from MDS

  • MDS should be confirmed by standard diagnostics and >3 mo prior to AML diagnosis

Progressed from MDS/MPN (specify type)

  • MDS/MPN should be confirmed by standard diagnostics and >3 mo prior to AML diagnosis

Germline predisposition (specify type)   

Classification adopted from reference 1.

BM, bone marrow; MPAL, mixed phenotype acute leukemia.

* Bone marrow or peripheral blood blast count of ≥ 10 % required, except for AML with t(9;22)(q34.1;q11.2)/BCR::ABL1 which requires bone marrow or peripheral blood blast count of ≥ 20% due to its overlap with progression of chronic myeloid leukemia, BCR::ABL1-positive.

† Other recurring translocations involving RARA should be reported accordingly: eg, APL with t(1;17)(q42.3;q21.2)/IRF2BP2::RARA; APL with t(5;17)(q35.1;q21.2)/NPM1::RARA; APL with t(11;17)(q23.2;q21.2)/ZBTB16::RARA; APL with cryptic inv(17) or del(17)(q21.2q21.2)/STAT5B::RARA; STAT3::RARA; other genes rarely rearranged with RARA: TBL1XR1 (3q26.3); FIP1L1 (4q12); BCOR (Xp11.4).

‡ Other recurring translocations involving KMT2A should be reported accordingly: eg, AML with t(4;11)(q21.3;q23.3)/AFF1::KMT2A; AML with t(6;11)(q27;q23.3)/AFDN::KMT2A; AML with t(10;11)(p12.3;q23.3)/MLLT10::KMT2A; AML with t(10;11)(q21.3;q23.3)/TET1::KMT2A; AML with t(11;19)(q23.3;p13.1)/KMT2A::ELL; AML with t(11;19)(q23.3;p13.3)/KMT2A::MLLT1.

* Other recurring translocations involving MECOMshould be reported accordingly: eg, AML with t(2;3)(p11∼23;q26.2)/MECOM::?; AML with t(3;8)(q26.2;q24.2)/MYC, MECOM; AML with t(3;12)(q26.2;p13.2)/ETV6::MECOM; AML with t(3;21)(q26.2;q22.1)/MECOM::RUNX1.

‖ Other rare recurring translocations: AML with t(1;3)(p36.3;q21.3)/PRDM16::RPN1; AML (megakaryoblastic) with t(1;22)(p13.3;q13.1)/RBM15::MRTFA; AML with t(3;5)(q25.3;q35.1)/NPM1::MLF1; AML with t(5;11)(q35.2;p15.4)/NUP98::NSD1; AML with t(7;12)(q36.3;p13.2)/ETV6::MNX1; AML with t(8;16)(p11.2;p13.3)/KAT6A::CREBBP; AML with t(10;11)(p12.3;q14.2)/PICALM::MLLT10; AML with t(11;12)(p15.4;p13.3)/NUP98::KMD5A; AML with NUP98 and other partners; AML with t(16;21)(p11.2;q22.2)/FUS::ERG; AML with t(16;21)(q24.3;q22.1)/RUNX1::CBFA2T3; AML with inv(16)(p13.3q24.3)/CBFA2T3::GLIS2.

¶ AML with in-frame mutation in the bZIP domain of the CEBPA gene, either monoallelic or biallelic.

Referenser

  1. Döhner H, Wei AH, Appelbaum FR, Craddock C, DiNardo CD, Dombret H, et al. Diagnosis and management of AML in adults: 2022 recommendations from an international expert panel on behalf of the ELN. Blood. 2022;140(12):1345-77.
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