one third will not require treatment and have a long survival
one third will have an initial indolent phase followed by disease progression
remaining third presents with aggressive disease and will need immediate treatment (1)
The median survival of patients with CLL at diagnosis differs between 1 and >10 years (2,3).
UK data (2):
chronic lymphocytic leukaemia mortality is strongly related to age, with the highest mortality rates being in older people
in the UK in 2014-2016, on average each year around three-quarters (74%) of deaths were in people aged 75 and over
largely reflects higher incidence and lower survival for chronic lymphocytic leukaemia in older people
age-specific mortality rates rise steeply from around age 60-64. The highest rates are in the 90+ age group for males and females. Mortality rates are significantly higher in males than females in a number of (mainly older) age groups. The gap is widest at age 50 to 54, when the age-specific mortality rate is 5.8 times higher in males than females
Although Rai and Binet staging systems are used to predict the prognosis in CLL, neither of the two systems can
identify patients with indolent or progressive disease
predict the response to treatment (4).
In addition to clinical staging, the following traditional factors have some value but also have important drawbacks (1)
short lymphocyte doubling time
diffuse bone marrow infiltration
high serum levels of b2-microglobulin
high serum levels of soluble CD23 (4)
Therefore newer prognostic markers have been proposed to identify risk of disease progression:
immunoglobulin variable region heavy chain gene (IgVH) mutation
in IgVH mutation - median survival is more than 20 to 25 years
in unmutated IgVH - the survival is 8 to 10 years
chromosomal abnormalities by fluorescent in situ hybridization (FISH)
with this method over 80% of chromosomal abnormalities in the CLL patients has been identified
deletion 13q -
the commonest cytogenetic abnormality (55%)
is associated with a favourable prognosis and longest survival
deletion of 11q
found in 18% of the patients
is associated with development of extensive lymphadenopathy and shorter survival (79 months)
deletion 17p13 -
has the poorest prognosis
is linked to shorter treatment free intervals, shorter survival (32 months), and resistance to therapy
ZAP-70 (an intracellular protein tyrosine kinase, which plays a critical role in T-cell receptor signalling)
in positive ZAP-70 - median survival is 6-10 years
in negative ZAP-70 - survival was more favourable (>15 years) (4)
CD38 immunophenotype
CD38 positivity (>30%) is associated with a worse prognosis (5)
it is important to note that the optimum cut off value is uncertain (1)
Note:
the presence of poor prognostic factors should not be used to start treatment earlier in asymptomatic patients (4)
5. National Cancer Institute at the National Institutes of Health. Chronic Lymphocytic Leukemia Treatment. Stage information for chronic lymphocytic leukemia
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