Treatment option with del 17 positive but IGHV mutated

[Ashwas]

I am CLL since 2003 on wait and watch. Now my count has increased to 80000 and platlets fallen to 1 Lakhs. RBC is normal.My spleen is 16.5 cms

My FISH report indicates del17 positive but IGHV is mutated.

Is my CLL agressive and needs special treatment especially because I am del17 positive . But IGHV is mutated.

I consulted doctor and he feels that eventhough treatment is not immediately required, I may be requiring it.

What may be first line treatment for me

[lankisterguy Volunteer]

Hi Ashwas,-

Since you are 17p deleted I believe all CLL expert doctors would avoid using any traditional chemotherapy (no Chlorambucil, Bendamustine, Fludarabine or Cyclophosphamide).

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Depending on the drugs available to your doctors in India, Ibrutinib / Imbruvica might be the suggested choice economictimes.indiatimes.co...

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(or Venetoclax taken after another drug like Ibrutinib / Imbruvica or Rituxan is used to reduce your spleen and lymph nodes).

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BTW, we have a few members that live in India- you may be able to find them by using this feature healthunlocked.com/people-n...

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Len

[cajunjeff]

Ashwas, we have all sorts of markers that predict the course of our Cll, FISH and IGHV status being two of the most important. Most people with 17p Cll also have unmutated ighv, both of which predict a more aggressive type of Cll.

In your case, your markers are considered discordant, that is, one good and one bad. Generally speaking, from what I have read (and I am no expert), those with 17p and mutated IGHV Cll do better than those with 17p and unmutated IGHV. Your IGHV status should not change so that’s good too.

You have a lot of good treatment options not widely available ten years ago. They don’t include chemo because of your 17p, but there is a move away from chemo for most everyone these days.

And markers are no guarantee of how our Cll progresses, some with good markers have aggressive Cll and some with worse markers go years without needing treatment.

They are trying to figure out which combination of new drugs work best for us. If you needed treatment in the near future, a safe bet is just a btk drug by itself like ibrutinib or acalabrutinib that do very well with 17p Cll as a first treatment.

[Ashwas in reply to cajunjeff]

Thanks a lot for the replyYour reply is really assuring

Initially at the time of diagnosis, I was told there is no genetical abnormality. However, the latest FISH report it indicates 12% del 17 observed. What is the cut off percentage.

Even in the IGHV test, they have not clearly replied the type of treatment .

Is checking positivity of CD 38 help in predicting the prognosis of the disease.

[cajunjeff in reply to Ashwas]

I don’t think there is a clear consensus of what the cut off percentage is to be considered 17p positive. I have seen it as high as 20 to 25 % with other studies suggesting a 10 cut off and some even lower than that. At 12% 17p cells you might not even be considered 17p positive.

I do not know what you mean by your IGHV test not discussing treatment. Treatment suggestions would not typically be included in the results of a IGHV mutation test report.

While it’s natural to worry about treatment decisions at any stage of our cll, since it doesn’t sound like treatment is imminent for you I don’t know if I would spend a lot of energy worrying about it. The preferred treatment for Cll might be different a few years from now.

If you were to treat now your options would likely be ibrutinib, acalabrutinib, venetoclax or obinutuzumab as single agents or in some sort of combination therapy.

Being CD 38 positive is another marker that predicts aggressive Cll. The best predictor of how aggressive our Cll is, is how our Cll actually acts. If you have been on watch and wait 18 years, your Cll seems indolent thus far.

[Ashwas in reply to cajunjeff]

Thanks for the informative reply. Now I am relaxed and will wait how the things get unfold in future. You have rightly pointed that new drugs are coming with every passing period.

[AussieNeil Administrator in reply to Ashwas]

Ashwas, prognostic markers are only statistically relevant to groups of people with markers in common. For an individual, what really counts is our measured rate of progression in our blood counts. This pinned post covers the triggers for starting treatment: healthunlocked.com/cllsuppo...

The reason for this is well illustrated by considering the influence of IGHV and CD38 status on progression. Those who are CD38 negative have around a 60% chance of being mutated IGHV. IGHV mutation status rarely changes, but CD38 status can change over time. They are both considered independent predictors of time to treatments.

Also, given very few of us are familiar with lakhs units, it would help if you gave both your blood counts and the reference range for them. I think the reference range for your platelets is something like 1.5 to 4.5 lakh. One trigger for beginning treatment in the iWCLL Guidelines used to be platelets falling below 1 lakh. Per the latest iWCLL Guidelines update, treatment for you can be deferred provided your platelet count remains stable, even if it falls below 1 lakh.

Neil

[thompsonellen]

Hi, I'm also 17p deleted, mutated and I also carried the disease in my lymph nodes (SLL). I went from feeling fine to very sick within 2 months (and internal bleeding that required hospitalization, blood transfusion, etc.). My WBC was only 60K when I started treatment. I did a clinical trial (thanks to the blood transfusion which propped my numbers up high enough to qualify), but if I hadn't I would have been put on ibrutinib. Today, they might have put me on acalabrutinib. Please watch the progression of your disease. It can spin out of control very quickly with 17p, so if you start feeling fatigued (trouble walking up steps) you need to push for treatment ASAP. Ellen