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INTRODUCTION

Epidemiology

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Epidemiology
Incidence:

19,940 (male: 11,090; female: 8850. Estimated new cases for 2020 in the United States)

4.3 per 100,000 male and female per year

Deaths: Estimated 11,180 in 2020 (male: 6470; female: 4710)
Median age: 68 years (median age for acute promyelocytic leukemia: 40 years)
Male to female ratio: 1.48

 

Dores GM. Blood 2012;119:34–43

Siegel R et al. CA Cancer J Clin 2020;70:7–30

Surveillance, Epidemiology and End Results (SEER) Program, available from http://seer.cancer.gov (accessed in 2020)

Work-up

  • H&P

  • CBC and leukocyte differential counts, platelets, electrolytes, liver function tests, PT, PTT, INR, fibrinogen, LDH, uric acid

  • Bone marrow biopsy with cytogenetics (karyotype and FISH), immunophenotyping, and molecular studies (including c-KIT, FLT3 [ITD and TDK], NPM1, CEBPA [biallelic], IDH1, IDH2, TP53)

  • HLA typing for patients who are candidates for allogeneic hematopoietic stem cell transplantation

  • Chest x-ray and CT/MRI scans if clinically indicated to rule out infection or myeloid sarcoma

  • Echocardiogram if therapy with anthracycline is planned, especially if prior cardiac history or prior anthracycline use

  • Lumbar puncture if neurologic symptoms (LP should be performed if a mass/lesion is not detected on imaging studies)

Pathology

WHO Classification of Acute Myeloid Leukemia

  1. AML with recurrent genetic abnormalities

    • AML with t(8;21)(q22;q22) (RUNX1-RUNX1T1)

    • AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22) (CBFB-MYH11)

    • Acute promyelocytic leukemia with PML-RARA

    • AML with t(9;11)(p21.3;q23.3); MLLT3-KMT2A

    • AML with t(6;9)(p23;q34.1); DEK-NUP214

    • AML with inv(3)(q21q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM (EVI1)

    • AML (megakaryoblastic) with t(1;22)(p13.3;q13.3); RBM15-MKL1

    • Provisional entity: AML with BCR-ABL1

    • AML with mutated NPM1

    • AML with biallelic mutations of CEBPA

    • Provision entity: AML with mutated RUNX1

  2. AML with MDS-related changes

  3. Therapy-related myeloid neoplasms

  4. AML not otherwise specified

    • AML minimal with differentiation

    • AML without maturation

    • AML with maturation

    • Acute myelomonocytic leukemia

    • Acute monoblastic/monocytic leukemia

    • Pure erythroid leukemia

    • Acute megakaryoblastic leukemia

    • Acute basophilic leukemia

    • Acute panmyelosis with myelofibrosis

  5. Myeloid sarcoma

  6. Myeloid proliferation related to Down syndrome

    • Transient abnormal myelopoiesis (TAM)

    • Myeloid leukemia associated with Down syndrome

Blastic plasmacytoid dendritic cell neoplasms

Acute leukemias of ambiguous lineage

  • Acute undifferentiated leukemia

  • Mixed phenotype acute leukemia with t(9;22)(q34.1;q11.2); BCR-ABL1

  • Mixed phenotype acute leukemia with t(v;11q23.3); KMT2A rearranged

  • Mixed phenotype acute leukemia, B/myeloid, not otherwise specified

  • Mixed phenotype acute leukemia, T/myeloid, not otherwise specified

Myeloid neoplasms with germ-line predisposition without a pre-existing disorder or organ dysfunction

  • AML with germ line CEBPA mutation

  • Myeloid neoplasms with germ line DDX41 mutation

Myeloid neoplasms with germ line predisposition and pre-existing platelet disorders

  • Myeloid neoplasms with germ line RUNX1 mutation

  • Myeloid neoplasms with germ line ANKRD26 mutation

  • Myeloid neoplasms with germ line ETV6 mutation

Myeloid neoplasms with germ line predisposition and other organ dysfunction

  • Myeloid neoplasms with germ line GATA2 mutation

  • Myeloid neoplasms associated with bone marrow failure syndromes

  • Juvenile myelomonocytic leukemia associated with neurofibromatosis, Noonan syndrome, or Noonan syndrome–like disorders

  • Myeloid neoplasms associated with Noonan syndrome

  • Myeloid neoplasms associated with Down syndrome

Arber DA et al. Acute myeloid leukaemia with recurrent genetic abnormalities. In: Swerdlow S et al. editors. ...

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