3.2 Prognostic assessment

3.2 Prognostic assessment

Staging is a critical element in treatment planning and should be clearly documented in the patient’s medical record.

Staging for CLL involves a physical exam and complete blood count.

In Australia, the Rai staging system is most often used to determine the stage of CLL (the Binet staging system is more commonly used in some other regions). For the purposes of this OCP, we will use the Rai staging system.

Rai system

Low risk:

  • Rai 0 – an isolated peripheral blood lymphocytosis > 5 × 109/L Intermediate risk:
  • Rai I – lymphocytosis and lymphadenopathy on clinical examination
  • Rai II – lymphocytosis and hepatomegaly and/or splenomegaly with/without lymphadenopathy High risk:
  • Rai III – lymphocytosis and haemoglobin < 110 g/L (6.83 mmol/L) with/without lymphadenopathy/ organomegaly
  • Rai IV – lymphocytosis and platelets < 100 × 109/L with/without lymphadenopathy/organomegaly

The ESMO guidelines define Rai 0 as low risk, Rai I and II as intermediate risk, and Rai III/IV as high risk. But it may be more useful to consider CLL in terms of early-stage disease: Rai 0 to I, and late-stage disease, Rai II to IV (Eichhorst et al. 2021).

Newer prognostic models such as the CLL International Prognostic Index  can identify patients with high-risk disease who may benefit from investigational therapy, as well as those who have a good prognosis despite advanced stage (Hallek et al. 2018).

Prognostic markers include:

  • IGHV mutational status
  • serum beta-2 microglobulin
  • presence of del(17p) and/or TP53 mutations – these are strong determinants of high-risk