July 2018

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Bloodline July 2018

MONTHLY QUIZ: Concerning the reason we got CLL:

1. CLL can be familial, but that is rare.
2. CLL incidence is increased in those exposed to Agent Orange in Vietnam
3. CLL incidence is increased in those exposed to radiation from Chernobyl
4. CLL is linked to benzene exposure
5. All of the above
6. 1, 2 and 3 are correct.

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 in 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.

NEWS:

June, 2018: FDA approved venetoclax with rituximab as a fixed duration therapy for CLL patients for who have received 1 prior therapy. This is the 1^st CLL fixed duration non-chemo therapy approved. Also approved: venetoclax as monotherapy in those with or without 17p del who have received 1 prior therapy.

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 and mammograms and colonoscopies and skin check- catch the cancer early.

But in CLL there is no 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:

1. Until recently, all treatment options were either relatively toxic or ineffective.
2. Some patients will never need treatment so treating only exposes them to toxicities with no benefits

This may change for some patients. There are trials looking at early intervention with novel therapies for patients at high risk to progress. Outside of a clinical trial, watch and wait is still the smartest option.

ACRONYM OF THE MONTH

TKI: TKIs or tyrosine kinase inhibitors are drugs that work by blocking activation of different proteins. They inhibit signaling pathways, preventing the expected biochemical event in the cell. Ibrutinib is a TKI approved to treat CLL and there are others in development and still more used to treat other cancers.

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