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Over the course of a year of monthly meetings, The CLL Bloodline will teach the BASICS needed to understand CLL, starting this month with definitions and diagnosis. It will also provide news, help with the acronyms and new vocabulary words, and offer simple fun quizzes.
Chemo-immunotherapy (CIT), specifically FCR (fludarabine, cyclophosphamide, and rituximab), can give very long remissions that are starting to look like cures when used as frontline therapy for some CLL patients who:
1: Are missing the short arm of chromosome 17, also known as deletion 17p
2: Have mutated IGVH (correct)
3: Have unmutated IGVH
4: We can’t predict who is more likely to respond to CIT
FCR had resulted in extremely durable remissions for some low-risk patients. Quoting from the journal Blood, January 2016: The 12.8-year PFS (progression free survival) was 53.9% for patients with mutated immunoglobulin heavy chain variable (IGHV) gene (IGHV-M) and 8.7% if unmutated IGHV (IGHV-UM).
That almost 13 year survival rate is not showing any drop off. It has plateaued with no more folks progressing and the data is now out almost 15 years. In fact for healthy patients with the best prognostic factors, especially if mutated (IGVH-M), if FCR gets them to where there is no measurable disease (MRD or minimal residual disease negative), then they have a greater almost an 80% chance of never needing any more treatment for CLL. Mutated CLL is good news as the mutated CLL cells are more mature and tend to grow more slowly. However, if one does not fit into the low risk group, odds with FCR are much worse. FCR or any chemo should never be used for those with deletion 17p. In summary, there is a very small group for whom FCR still makes sense as their 1st treatment, but in the light of all the new therapies, most have better options.
On Jan. 22, 2018, Celgene announced they had acquired Juno Therapeutics. Celgene is a large pharmaceutical company with a strong presence in blood cancers, and while it has no CLL drugs, it does have a promising pipeline for CLL. Juno is a newer smaller company that is focused exclusively on the development of CAR-T and related therapy to treat different cancers, including CLL. Juno’s newest CAR-T, JCAR017, looks very promising in CLL.
THE BASICS: What to do when first diagnosed:
CLL is a slow growing or indolent lymphoma of the B-lymphocytes and gives you time to plan your therapy. Don’t neglect your other medical care especially age and gender appropriate cancer screening as CLL increases the risk of secondary cancers. Get up to date with vaccinations, but avoid all live vaccines such as the old shingles or yellow fever as they are not known to be safe for those with CLL. Most importantly, put together you team (see the CLL Society online toolkit for help), join a support group and learn more about your disease.
WORD/ACRONYM OF THE MONTH: (2 words and one acronym this month)
Petechiae: Small red or purple pinhead spots on the skin. They are caused by bleeding under the skin and are usually the result of a shortage of platelets. They are similar to purpura, but are much smaller in size. In CLL, some patients’ immune system destroys their own platelets. This is called ITP or Idiopathic (or Immune) Thrombocytopenic Purpura in which the body’s dysfunctional immune system attacks its own platelets which results in bruising including petechiae and purpura and/or bleeding.