ESH 2014: Prof. Stephan Stilgenbauer Reviews Predictive and Prognostic Markers in CLL (chronic lymphocytic leukemia)
As a result of the positive response I received for my 30 minutes on the podium, I was asked if I could shoot a series of monologues that address various aspects of why it is so critical that our voices be heard by the professional community that treats us. I will posting those here and later our CLL website soon. Check out the first monologue here filmed soon after I flew home. More monologues to follow on other topics.
The Hellenic hematology congress was co-incident with another valuable meeting in Thessaloniki organized by the European School of Hematology (ESH), ESH 2014: International Conference on New Concepts in B Cell Malignancies: From molecular pathogenesis to personalized treatment so I stayed in town for an extra few days to attend the CLL sessions and shoot some videos for the blog.
On a personal note, on my one half day free in Macedonia, I did rent a car and drove in the rain to see the amazing underground museum that includes the unspoiled grave of Philip II, father of Alexander the Great. Don't miss it. I loved my much too brief return visit to Greece.
The first video interview is with Prof. Dr. med. Stephan Stilgenbauer of the University of Ulm.
We revisit the topic of prognosis and predictive factors. Here is a link to an earlier post on another aspect of this subject with Dr. Bill Wierda from MD Anderson. Dr. Jeff Sharman gets into some of the same material in this helpful post from iwCLL 2013.
The first message that I gathered from Prof. Stilgenbauer is that the significance of all these factors must be reassessed in this era of novel therapies and that process has only just begun. How they may have predicted and prognosticated about treatment with FCR is not always going to match up as to their relevance for therapy with ibrutinib or idelalisib.
This should be an active area or reassessment and research. One possible example seems to be that patients with 17p deletion respond differently when treated upfront with ibrutinib versus when treated after they have relapsed with 17p deletion. More predictive and prognostic factors will emerge if we look.
My next take way: It is no longer sufficient to test only only for the deletion of the short (p for petite) arm of the 17th chromosome (17p deletion), but we also must ask our hematologists to check the function of the TP53 gene. TP53 has been called the guardian of the genome because when it is functioning well, it protects our genetic information from being miscopied and thus spawning a malignant offspring. When it is missing or dysfunctional, cancers are both meaner and harder to kill. Here is a nice overview on TP53 from the NIH. Dr. Stilgenbauer discusses the relation between TP53 and 17p deletion.
Finally we touch briefly on some of the new prognostic factors found in CLL with the advent of deeper probing of our cancer's genetic makeup with next generation sequencing. These include mutations in NOTCH1 (a signaling pathway between adjacent cells) and SF3B1 (a surprise finding, a important in gene splicing). Here's the original article from the NEJM that goes into much greater detail.
Here is Dr. Stilgenbauer.
More soon from ESH and ASH.
As I write and edit these recent blog posts, it has become increasingly clear to me that the scope of what needs to be done to teach about CLL had grown beyond the capacity of this meager blog.
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