MONTHLY QUIZ: Concerning the reason we got CLL, choose the correct statement below:
- CLL can be familial, but that is rare.
- CLL incidence is increased in those exposed to Agent Orange in Vietnam.
- CLL incidence is increased in those exposed to radiation from Chernobyl.
- CLL is linked to benzene exposure.
- All of the above.
- 1, 2 and 3 are correct.
ANSWER: The correct answer is #6.
CLL mostly occurs episodically with no known cause, but a small percentage of CLL runs in families. Agent Orange is a recognized risk for CLL and exposed veterans who develop CLL maybe entitled to compensation. For a long time, radiation was not considered a risk due to the lack of increase of CLL after Hiroshima, but we now know from the Chernobyl experience, that Hiroshima was the exception due to the very low baseline incidence of CLL in Japanese. Benzenes and other solvents may increase the risk of other blood cancers, but there has been no link found with CLL. Usually we just don’t know why we got CLL.
THE BASICS: Watch and Wait
Watch and Wait or Active Observation, or as patients often call it, Watch and Worry is at first glance one of the most counter-intuitive concepts in CLL management. With many types of cancer early detection is everything and the prognosis gets worse with more advanced stages of the disease. That is the whole philosophy behind regular PAP smears, mammograms, colonoscopies, and skin check—catch the cancer early.
But in CLL until the study reported above, data showing that earlier treatment at the time of diagnosis helps, and in fact there is some old data that it doesn’t. There are two main reasons for this:
- Until recently, all treatment options were either relatively toxic or ineffective.
- Some patients will never need treatment so treating only exposes them to toxicities with no benefits.
This may change for some patients with this new data, but with treatment moving towards fixed duration with novel agents, the place for this early intervention approach is controversial. Why take a medicine for years that you may never need and has not yet been shown to improve overall survival when taken early? Is taking it when needed just as good? Outside of a clinical trial, watch and wait is still the smart option.
June 2019: At the EHA (European Hematology Assoc.) Annual Congress, for the 1st time a clinical trial that compared ibrutinib to placebo in asymptomatic early stage high risk patients proved that ibrutinib significantly improves progression free and event free survival and time to next treatment. There were no significant differences in adverse events between the two groups.
ACRONYM OF THE MONTH: TKI
TKIs or tyrosine kinase inhibitors are drugs that work by blocking activation of different proteins. In CLL, they inhibit signaling pathways, preventing the cancer cell from communicating with other cells. Ibrutinib, idelalisib, and duvelisib are oral TKIs approved to treat CLL and others are in development.
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