Over the course of a year of monthly meetings, the CLL Bloodline will teach the BASICS needed to understand CLL, bring news, help with the acronyms and new vocabulary, and offer simple fun quizzes.
MONTHLY QUIZ: Having deletion 17p confirms a poor prognosis. Choose all that are correct:
- It is detected by “FISH” testing.
- It refers to missing the short arm of the 17th chromosome that should contain the important anti-cancer gene, TP53.
- It is associated with resistance to traditional chemotherapy, but less so with most novel agents.
- It interferes with the cells ability to commit suicide even when it is badly damaged.
- It can lead to increased genomic instability.
- It is common at time of diagnosis but becomes rarer as the CLL progresses.
Correct answer is all are true, but the last. Deletion 17p (del 17p) is detected by an important prognostic test called FISH testing (fluorescent in-situ hybridization) that probes the inside of the cells to look for missing or extra genetic material. The short or the petit arm of the 17th chromosome contains P53, a potent anti-cancer gene that has been called the guardian of the genome. P53 tries to repair damaged DNA and if it can’t repair the damage, it starts the process for the cell to die. Without P53, cells damaged by chemotherapy are not killed, but instead continue to reproduce and may become even more mutant and resistant to therapy. Del 17p is only detectable in 5% at diagnosis but is found in 30% or more at relapse. It can develop without treatment. That is why we recommend FISH testing before starting each line of therapy, as traditional chemo will not work for those with del 17p. TEST BEFORE TREAT™ is our mantra at the CLL Society.
NEWS: CLL Society is leading a federal and international public and behind the scenes effort to recognize the needs of the immunocompromised in the pandemic, and to prepare for the next. Dr. Koffman was quoted by CNN, referring to the new double dosing of Evusheld: “It doesn’t change the fact that many of the immunocompromised can still greatly benefit from pre-exposure prophylaxis.”
THE BASICS: When is treatment needed? The decision to start treatment should never be based on blood counts alone, but by looking at the whole patient. Consider initiating therapy when there is the presence of:
- B Symptoms
- Weight loss >10% of body weight in previous 6 months
- Severe fatigue (ambulatory and capable of all self-care but unable to carry out any work activities)
- Fevers >38°C for at least 2 weeks without evidence of infection
- Drenching night sweats for more than a month without evidence of infection
- Evidence of progressive bone marrow failure manifest by low blood counts (cytopenias) including anemia (low red blood cells) or thrombocytopenia (low platelets)
- Massive or symptomatic splenomegaly (enlarged spleen)
- Massive or symptomatic lymph nodes or clusters of nodes (>10 cm)
- Autoimmune Hemolytic Anemia (AIHA: body attacks its own red cells) and/or Immune Thrombocytopenic Purpura (ITP: body attacks its own platelets) that is unresponsive to steroids or other standard therapy
- Rising ALC with an increase of more than 50% over a 2-month period or a lymphocyte doubling time (LDT) <6 months. If ALC is <30,000, LDT should not be used as the only criterion for beginning to treat.
See http://cllsociety.org/2016/03/cll-watch-wait-start-treatment/ for more discussion of this important topic and the related subject of watch and wait. Remember that there is no lymphocyte count that says it’s time to treat!
WORD/ACRONYM OF THE MONTH: Tumor Lysis Syndrome (TLS)
Tumor Lysis Syndrome is a complication of treatment caused by the rapid killing of the cancer cells that can result in dangerously high uric acid and potassium blood levels that can lead to serious heart and kidney issues. It may occur with oral meds such as venetoclax and IV treatments including CAR-T when the leukemia cells are lysed (killed) too fast and spill out their inner contents into the blood stream, overwhelming the kidneys’ ability to eliminate the excess products of the cellular breakdown.