Staging of chronic lymphocytic leukaemia

Two clinical staging systems are used widely. (1)

• the Binet staging system - used in Europe,
  • is based on the
    • number of involved areas - defined by the presence of enlarged lymph nodes of greater than 1 cm in diameter or organomegaly in the following areas
      • head and neck, including the Waldeyer ring (this counts as one area, even if more than one group of nodes is enlarged)
      • axillae (involvement of both axillae counts as one area).
      • groins, including superficial femorals (involvement of both groins counts as one area).
      • palpable spleen
      • palpable liver (clinically enlarged)

and

• the presence of anaemia or thrombocytopenia
• it separates patients into three groups of different prognosis
  • Binet A
    • Hb ≥10.0 g/dl, thrombocytes ≥100×109/l, <3 lymph node regions involvement
    • median survival is >10 years
  • Binet B
    • Hb ≥10.0 g/dl, thrombocytes ≥100×109/l, ≥3 lymph node regions involvement
    • median survival is 7 years
  • Binet C
    • Hb <10,0 g/dl, thrombocytes <100×109/l
    • median survival is 1.5–2.5 years
• Rai staging system - used in the United States, named after its originator, this classification categorises patients in terms of how extensive the disease is at the time of presentation
  • low risk
    • Rai 0 - Lymphocytosis >15×109/l, median survival is >10 years
  • intermediate risk
    • Rai I - Lymphocytosis and lymphadenopathy
    • Rai II - Lymphocytosis and hepatomegaly and/or splenomegaly with/without lymphadenopathy
    • median survival is 7 years
  • high risk
    • Rai III - Lymphocytosis and Hb < 11.0 g/dl with/without lymphadenopathy/ organomegaly
    • Rai IV- Lymphocytosis and thrombocytes < 100×109/l with/without lymphadenopathy/ organomegaly
    • median survival is 1.5 - 3 years

Note:

• although these staging systems predicts prognosis, they are unable to identify who has indolent or progressive disease or predict response to treatment

Reference:

1. Hallek M, Al-Sawaf O. Chronic lymphocytic leukemia: 2022 update on diagnostic and therapeutic procedures. Am J Hematol. 2021 Dec 1;96(12):1679-705.