Criteria

Rai staging system[3]

  • 0 = Clonal lymphocytosis (>5 × 10⁹/L [>5000 cells/microlitre]) in blood and/or >40% lymphocytes in bone marrow (low risk)

  • I = Lymphocytosis plus lymphadenopathy (intermediate risk)

  • II = Lymphocytosis plus hepatomegaly and/or splenomegaly, with or without lymphadenopathy (intermediate risk)

  • III = Lymphocytosis plus anaemia (haemoglobin <110 g/L [<11 g/dL] or haematocrit <33%, with or without organomegaly (high risk)

  • IV = Lymphocytosis plus thrombocytopenia (platelets <100 × 10⁹/L [<100,000/microlitre]), with or without anaemia or organomegaly (high risk)

Binet staging system[50]

  • A = Haemoglobin ≥100 g/L (≥10 g/dL) and platelets ≥100 × 10⁹/L (≥100,000/microlitre) and <3 lymphoid areas enlarged (low risk)

  • B = Haemoglobin ≥100 g/L (≥10 g/dL) and platelets ≥100 × 10⁹/L (≥100,000/microlitre) and ≥3 lymphoid areas enlarged (intermediate risk)

  • C = Presence of anaemia (haemoglobin <100 g/L [<10 g/dL]) and/or thrombocytopenia (platelets <100 × 10⁹/L [<100,000/microlitre]) with any number of lymphoid areas enlarged (high risk)

Chronic lymphocytic leukaemia international prognostic index (CLL-IPI)[40]

An international prognostic index for CLL developed by the CLL-IPI working group. The index combines genetic, biochemical, and clinical parameters into a prognostic model to stratify patients into four risk groups (low, intermediate, high, and very high).

The five prognostic factors used in the index are:

  • TP53 status (no abnormalities vs. del(17p) and/or TP53 mutation)

  • Immunoglobulin heavy chain (IgHV) mutational status (mutated vs. unmutated)

  • Serum beta2-microglobulin concentration (≤3.5 mg/L vs. >3.5 mg/L)

  • Clinical stage (Binet A or Rai 0 vs. Binet B to C or Rai I-IV)

  • Age (≤65 vs. >65 years)

The CLL-IPI utilises modern prognostic factors and may provide improved prognostication in CLL; however, it requires further evaluation in prospective trials.

International workshop on chronic lymphocytic leukemia (iwCLL) treatment response criteria[2]

Complete remission (all of the criteria have to be met)

  • Group A parameters

    • Lymph nodes: none ≥1.5 cm

    • Liver and/or spleen size: spleen size <13 cm; liver size normal

    • Constitutional symptoms: none

    • Circulating lymphocyte count: normal

  • Group B parameters

    • Platelet count: ≥100 × 10⁹/L

    • Haemoglobin: ≥110 g/L (≥11 g/dL; untransfused and without erythropoietin)

    • Marrow: normocellular, no CLL cells, no B-lymphoid nodules

    • Neutrophil count: ≥1.5 × 10⁹/L

Partial remission (at least 2 of the parameters of group A and 1 parameter of group B need to improve if previously abnormal; if only 1 parameter of both groups A and B is abnormal before therapy, only 1 needs to improve)

  • Group A parameters

    • Lymph nodes: decrease ≥50% from baseline (sum of the products of 6 or fewer lymph nodes as evaluated by CT scans and physical examination in clinical trials or by physical examination in general practice)

    • Liver and/or spleen size: decrease ≥50% from baseline

    • Constitutional symptoms: any

    • Circulating lymphocyte count: decrease ≥50% from baseline

  • Group B parameters

    • Platelet count: ≥100 × 10⁹/L or increase ≥50% over baseline

    • Haemoglobin: ≥110 g/L (≥11 g/dL) or increase ≥50% over baseline

    • Marrow: presence of CLL cells, or of B-lymphoid nodules, or not done

Progressive disease (at least 1 of the criteria of group A or group B has to be met)

  • Group A parameters

    • Lymph nodes: increase ≥50% from baseline or from response

    • Liver and/or spleen size: increase ≥50% from baseline or from response

    • Constitutional symptoms: any

    • Circulating lymphocyte count: increase ≥50% over baseline

  • Group B parameters

    • Platelet count: decrease of ≥50% from baseline secondary to CLL

    • Haemoglobin: decrease of 20 g/L (≥2 g/dL) from baseline secondary to CLL

    • Marrow: increase of CLL cells by ≥50% on successive biopsies

Stable disease (all of the criteria have to be met; constitutional symptoms alone do not define progressive disease).

  • Group A parameters

    • Lymph nodes: change of -49% to +49%

    • Liver and/or spleen size: change of -49% to +49%

    • Constitutional symptoms: any

    • Circulating lymphocyte count: change of -49% to +49%

  • Group B parameters

    • Platelet count: change of -49 to +49%

    • Haemoglobin: increase 110 g/L (<11 g/dL) or <50% over baseline, or decrease <20 g/L (<2 g/dL)

    • Marrow: no change in marrow infiltrate

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