Leukemias 

Leukemias

 

 

Definition:
Leukemias are categorized into chronic and acute forms:

 

Chronic Leukemias:

Chronic lymphocytic leukemias (CLL)  (adapted from Bennett JM, et al. J Clin Pathol 42:567, 1989)

  • B cell lymphocytic leukemias
    • Small B cell chronic lymphocytic leukemia (CLL, B-CLL)
    • Mantle cell leukemia (MCL)
    • Hairy cell leukemia (HCL) 
    • Prolymphocytic B cell leukemia (B-PLL)
    • Leukemic phase of splenic lymphoma with villous lymphocytes (SLVL)
    • Leukemic phase of marginal zone lymphoma (MZL)
    • Leukemic phase of lymphoplasmacytic lymphoma (LPL) including Waldenstrom's macroglobulinemia (WM)
    • Leukemic phase of follicular lymphoma (leukolymphosarcoma)
  • T and NK cell lymphocytic leukemias
    • Large granular lymphocyte (LGL) syndrome (leukemia)
    • Leukemic phase of mycosis fungoides (MF) and Sezary's syndrome (SS)
    • Prolymphocytic T cell leukemia (T-PLL)
    • Small T cell chronic lymphocytic leukemia (T-CLL)

Chronic myelocytic leukemias (CML) 

  • Chronic myelocytic leukemia- classified under "myeloproliferative disorders"
  • Chronic myelomonocytic leukemia- classified under "hybrid MPD-MDS disorders"

Acute Leukemias:

Acute lymphocytic (lymphoblastic) leukemias (ALL) 

FAB Classification: (adapted from Bennet JM, et al. Br J Haematol 47:553, 1981)

Other variants:

WHO Classification (adapted from Swerdlow SH, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2008, p167)

  • Precursor lymphoid neoplasms
    • Precursor B lymphoblastic leukemia/lymphoma, NOS
    • B lymphoblastic leukemia/lymphoma with recurrent genetic abnormalities
      • B lymphoblastic leukemia/lymphoma with t(9:22)(q34;q11.2); BCR-ABL1
      • B lymphoblastic leukemia/lymphoma with t(v;11q23); MLL rearranged
      • B lymphoblastic leukemia/lymphoma with t(12;21)(p12;q22); TEL-AML1 (ETV6-RUNX1)
      • B lymphoblastic leukemia/lymphoma with hyperdiploidy
      • B lymphoblastic leukemia/lymphoma with hypodiploidy
      • B lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32); IL3-IGH
      • B lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); E2A-PBX1(TCF3-PBX1)
    • Precursor T lymphoblastic leukemia/lymphoma

    Acute myeloid (myelogenous, myelocytic, myeloblastic, granulocytic, nonlymphoid) leukemia (AML)

    FAB Classification: (adapted from Bennet JM, et al. Ann Intern Med 103: 620, 1985)

    • M0 (AML with minimal differentiation, often with a bilineage immunophenotype)
    • M1 (acute myeloblastic leukemia without differentiation)
    • M2 (acute myeloblastic leukemia with differentiation)
      • M2Eo (with eosinophilic differentiation)
      • M2Ba (with basophilicdifferentiation)
    • M3 (acute promyelocytic leukemia, APML)
      • Hypergranular (M3)
      • Hypogranular (microgranular) variant (M3v)
    • M4 (acute myelomonocytic leukemia, Naegeli's acute leukemia, AMML)
      • M4Eo (with eosinophilic differentiation)
    • M5 (acute monocytic leukemia, Schilling's acute leukemia, AMoL)
      • M5a (undifferentiated)
      • M5b (AMoL with differentiation)
    • M6 (acute erythroid leukemia, erythroleukemia, erythemic myelosis, diGuglielmo's syndrome)
    • M7 (acute megakaryocytic leukemia)
      • micromegakaryoblastic variant
      • megakaryoblastic variant
    • Other terms or variants
      • Acute basophilic leukemia
      • Acute panmyelosis with myelofibrosis (acute myelofibrosis, acute myelosclerosis)- discussed under MPDs
      • Hypoplastic AML
      • Smoldering AML

    WHO Classification of AML (adapted from Swerdlow SH, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2008, p109)

    • AML with recurrent genetic abnormalities (adapted from Arber DA, et al. In Swerdlow SH, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2008, p110)
      • AML with the t(8;21)- most are M2 AMLs
      • AML with the INV(16) or t(16;16)- most are M4 AMLs with abnormal eosinophils
      • AML with the t(15;17)- M3 AML, APML
      • AML with 11q23 abnormalities- usually has monocytic features
      • AML with the t(9;11) (MLLT3-AML)
      • AML with 11q23 abnormalities
      • AML with the t(6;9) (DEK-Nup214)
      • AML with the INV3; t(3;3); RPN1-EV11
      • AML with the t(1;22); RBM15-MKL1
    • AML with specific gene mutations
      • FLT3 (fms-related gene mutations)
      • NPM1 (nucleophosmin) mutations
      • CEBPA (CCAAT/enhancer binding protein-alpha mutations)
    • AML with associated MDS changes
      • AML emerging from MDS or MPD
      • De Novo AML with multilineage dysplasia
      • M6 AML: 2 variants
        • M6a- mixed erythroid and myeloid blasts
        • M6b- pure erythroid blasts
    • AML, therapy-related (secondary acute leukemias)- Includes those associated with exposure to alkylating agents, topoisomerase type II inhibitors, other drugs, and radiation.
    • AML, not otherwise specified (NOS)- included are many of the FAB subtypes
    • Myeloid sarcoma (chloroma, granulocytic sarcoma, extramedullary myeloid tumor)
    • Myeloid proliferations related to Down Syndrome
    • Blastic plasmacytoid dendritic cell neoplasm (blastic NK cell leukemia/lymphoma, agranular CD4+, CD56+ hematodermic neoplasm (discussed under Lymphomas)
    • Acute leukemia of ambiguous lineage (adapted from Borowitz MJ, et al. In Swerdlow SH, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France; IARC Press, 2008, p150)
      • Acute undifferentiated leukemia (AUL; stem cell leukemia, AML, NOS)
      • Mixed phenotype acute leukemias (MPALs)
        • MPAL with the t(9;22), BCP-ABL1
        • MPAL with the t(v;11q23); MLL gene gene rearrangements
        • MPAL (B/myeloid, NOS)
        • MPAL (T/myeloid, NOS)
        • MPAL (rare types)
      • Acute leukemia, unclassifiable (AL, U)

     

    General References:

    1. Bennett JM, Catovsky D, Daniel MT, et al: Proposals for the classification of chronic (mature) B and T lymphoid leukemias. J Clin Pathol. 42:567, 1989
    2. Bennett JM, Catovsky D, Daniel MT, et al: French-American-British (FAB) Cooperative Group: The morphological classification of acute lymphoblastic leukemia- concordance among observers and clinical correlations. Br J Haematol 47:553, 1981
    3. Bennett JM, Catovsky D, Daniel MT, et al: Proposed revised criteria for the classification of acute myeloid leukemia: A report of the French-American-British Cooperative Group. An Intern Med 103:620, 1985
    4. Swerdlow SH, Campo E, Harris NL, et al. Precursor lymphoid neoplasms. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2008, p167
    5. Swerdlow SH, Campo E, Harris NL, et al. Acute myeloid leukemia and related precursor neoplasma. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2008, p109
    6. Arber DA, Brunning RD, Le Beau MM, et al. Acute myeloid leukemia with recurrent genetic abnormalities. In Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2008, p110
    7. Borowitz MJ, Béné M.-C, Harris NL, et al. Acute leukemias of ambiguous lineage. In Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: IARC Press, 2008, p150

     

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    Charles  E.  Hess,  M.D.,FACP

    Professor of Internal Medicine
    Department:  Medicine
    Division: Hematology/Oncology