Is CLL an “Old Man’s Disease”?
One of the often heard clichés is that CLL is an “old man’s disease”. As with all clichés, there is a smidgen of truth in it. A 2008 article in “Blood” looked at CLL patient profile in USA over the time period of 1973 – 2004.
- The single most common age group diagnosed with CLL was 70 -79 years old. Roughly 30% of the cases registered fell into this age bracket.
- 60% of the cases were men, a statistical difference that persisted over the whole time period of the study.
- However, another way of looking at the same statistics is that a goodly percentage of the patients were younger than 70 years and fully 40% were women. And I am dead certain all of the patients were young at heart. So there.
- Comparing the time frames 1980-84 and 2000-04, the number of registered CLL cases increased by 20%. But before you jump to conclusions, the authors point out that the number of CBC blood tests done as part of routine annual checkups has increased dramatically over the two decades and this may be the reason why we are now seeing more cases of CLL that would have gone undiagnosed in the earlier time frame. It is a case of finding more of them when you go looking for problems.
- Even with the higher level of routine monitoring of blood counts, the authors believe CLL is an under-reported cancer. The actual number of cases may be higher than reported by the national registry SEER. This may be due to the fact that majority of CLL patients are not treated at diagnosis. Local oncologists sending in their data to SEER registries may overlook counting patients that are in Watch & Wait mode.
As you can see from the table above, fully 20% of the patients diagnosed with CLL over this time period (1980 through 2004) were young people in the age range of 15-59. These guys are mere spring chickens as it were, still busy building careers, raising kids and hopefully saving money for a distant retirement.
That raises an interesting question: does CLL have a different face in younger patients? Is age at the time of diagnosis an important factor? Should we be more aggressive in doing all the prognostic testing in the case of younger patients? A Mayo Clinic abstract (below) at the recent ASH2009 conference addressed this question.
Influence of Age at Diagnosis On Utility of Prognostic Testing in Patients with CLL
Tait D. Shanafelt, M.D., Kari Rabe*, Neil E. Kay, MD, Clive S. Zent, MD, Diane F. Jelinek, PhD, Susan Schwager*, Deborah Bowen*, Susan L Slager, PhD, Megan Reinalda*, Curtis A. Hanson, MD and Timothy G Call, MD
Mayo Clinic, Rochester, MN
PURPOSE: Numerous clinical and biologic parameters are able to predict time to first treatment (TFT) and overall survival (OS) for patients with CLL. The utility of prognostic testing in CLL patients may vary based on age at diagnosis given the higher mortality from other causes among older individuals. The goals of the present study were to i) evaluate differences in natural history of CLL patients based on age at diagnosis, ii) compare survival to age-matched individuals in the general population, and iii) determine the age-stratified utility of prognostic testing.
METHODS:
All 2487 CLL/SLL patients diagnosed between January 1995 and June 2008 and cared for in the Mayo Clinic Division of Hematology were categorized by age at diagnosis (<55 years, 55-64 years, 65-74 years, or ≥75 years) and evaluated for differences in clinical characteristics, TFT, and OS using chi-square tests. IGHV gene mutation analysis, ZAP-70 status, CD38 status, and cytogenetics abnormalities by interphase FISH testing were performed as part of clinical and/or research studies using methods previously described by our group. Cox regression was used to evaluate the ability of prognostic parameters to predict clinical outcome stratified by age at diagnosis. Patient survival was compared to that of the age matched Minnesota population.
RESULTS: When grouped by age, 593 patients were age <55, 713 age 55-64, 748 age 65-74, and 433 age ≥75 at diagnosis. As of last follow-up, 268, 282, 296, and 108 patients in these age groups have been treated, respectively and 112, 171, 254, and 199 patients have died. Survival decreased as the age at diagnosis increased (p<0.001). Overall Survival of CLL patients was significantly shorter than that of the age-matched general population for patients age <55 years (p<0.001), 55-64 years (p<0.001), and 65-74 years (p<0.001) at diagnosis but not those ≥75 years (p=0.136). Among Rai stage 0 patients, survival was also shorter than that of the age-matched general population for CLL patients age <55 years (p<0.001), 55-64 years (p<0.001), and 65-74 years (p<0.001) but not those ≥ age 75 at diagnosis (p=0.07).
No statistically significant differences were observed in CD38 status, IGHV mutation status, ZAP-70 status or the frequency of common cytogenetic abnormalities on FISH based on age at diagnosis category. Consistent with prior reports, all of these parameters were powerful predictors of TFT and OS for the overall cohort on univariate analysis (all p<0.001 for both TFT and OS). Given the variation in TFT and OS based on age at diagnosis, we next evaluated the utility of these prognostic parameters for predicting TFT and OS in each age category after adjusting for stage. CD38, IGHV mutation, and ZAP-70 each predicted TFT independent of stage for all age groups (all p <0.05). FISH results predicted TFT independent of stage for patients age 55-64 and 65-74 however did not reach statistical significance for those age <55 (p=0.18) or ≥75 (p=0.07). The utility of these parameters for predicting OS independent of stage was less consistent and varied by age. IGHV predicted OS independent of stage for patients <age 55 (p<0.001), 55-64 (p=0.007), and 65-74 (p≤0.001) but was not a significant predictor of OS among those ≥75 (p=0.15). CD38, ZAP-70, and FISH each predicted survival independent of stage for only 2 of the 4 age categories (ZAP-70 for age <55 and 55-64; FISH for age <55 and 65-74; CD38 for 55-64 and ≥75).
CONCLUSIONS: Survival of CLL patients is shorter than that of the age-matched general population for individuals age <75 at diagnosis regardless of disease stage. In contrast, survival does not differ from the age-matched general population among CLL patients ≥age 75 at diagnosis. Prognostic testing using CD38, IGHV mutation, and ZAP-70 is useful for predicting TFT independent of stage for CLL patients of all ages (including those ≥age 75) but has less value for predicting OS particularly as the age at diagnosis increases. IGHV appeared to be the best predictor of OS independent of stage among all patients age <75. Unexpectedly, prognostic testing had little utility for predicting OS independent of stage among patients age ≥75. These findings have important implications regarding the use of prognostic tests in patients with CLL as well as the use of test results to select patients for clinical trials testing early treatment for asymptomatic individuals.
What These Statistics Mean for Your Life
Sometimes my eyes glaze over when I look at a block of writing like this and it is hard to get the punch-lines. Let me see if I can make it a little easier on your “aging” eyes. The chart below describes age distribution of the patients seen at Mayo Clinic over a 12 ½ year period.
As you would expect, just over 23% of patients were younger than 55 years when they were diagnosed – roughly the same as the country wide statistics reported by SEER. Bulk of the patients were in the three older age groups.
Patient Deaths Over Study Period
Mayo reports the number of patient deaths in each age group over this study period. The trend is clear. Older patients were more likely to have died during the study period. “Only” 18.9% of the youngest group died but a whopping 46% of patients who were over 75 years at diagnosis died during the study period.
Does this mean older patients pay a higher penalty of death due to CLL compared to younger folks? No!! When compared to a similar age cohort who did not have CLL, there was no statistical difference in the percentage of deaths in the over 75 group. In other words, CLL did not have much of an impact on overall survival for this oldest group.
It makes sense when you think about it. By the time people get to be 75 years old, they tend to accumulate a bunch of other health issues. Twelve years later, they are pushing 87 years and it is reasonable that slightly less than half (46%) of them are dead by then. For this group of patients CLL is just one more health issue to deal with, along with all the other issues such as heart disease, brittle bones, diabetes, other cancer etc.
Moral of the story seems to be this: if you are 75 years or older at the time of diagnosis of CLL, it is most likely not going to be a big deal in the sense of reduced life expectancy. If you are also blessed with a relatively smoldering variety of CLL, you may want to consider not doing anything too aggressive by way of therapy. Chances are good you will be able to “run out the clock” with kinder and gentler therapy interventions to hold the line as necessary and die peacefully in your bed on account of something other than CLL.
The game plan for you might be looking to maximize the quality of your life, enjoying each and every day that you have coming to you, keeping a wary eye out for all the other things that can go wrong as you age. All of us have to go someday, and chances are good you would have lived out your natural life span before CLL gets you.
Please bear in mind, I am talking in generalities here. Each individual is different and has different expectations. You might come from a family that typically live into their late nineties, in which case you would not be happy about kicking the bucket when you are several years short of that goal! I have a very young at heart 90+ year old friend who has no intention of dying – ever, if he can help it. On the other hand, my mom at 83 thinks she is ancient and chomping at the bit to get done with it. It is all a matter of your personal expectations, your zest for life.
Bitter Pill for Younger Patients
The story is dramatically different at the other end of the age spectrum. As the table shows, younger patients paid a huge penalty in terms of their reduced life expectancy. Compared to similar age cohorts they died at a younger age and this difference was statistically significant. Young guys who are 55 years at diagnosis would be only 67 years old 12 years later. You would not expect a fifth of 67 year olds (18.9%) to die! Heck, most of them would have just become eligible for Social Security and hitting the golf courses, but for CLL. Telling these guys CLL is a “good cancer” is adding insult to injury.
“Managing” this incurable cancer requires savvy gamesmanship in younger patients. For starters, they have a lot more responsibilities to juggle as they struggle with CLL. Jobs, money, medical insurance, taking care of younger children – all of these are bigger issues when you are in the prime of your life. Dealing with CLL on top of everything else can get to be a real challenge.
Fortunately younger patients are also in better shape to handle more aggressive therapy since they are generally healthier and have fewer secondary health complications. Chances are good that they will not be able to nickel and dime this disease with low impact therapy and thereby run out the clock and live their normal life expectancy. For very young patients, the million dollar question is this: when (not if) to go into a mini-allo stem cell transplant. To date, this is the only technology known to CURE CLL. Every other therapy out there is able to do no more than give you breathing room – while you get your ducks in a row for an allogeneic transplant. The other choice is dying a lot sooner than you would have without the CLL. Sometimes it is a case of deciding between the devil and the deep blue sea. The good news is that transplant statistics are improving each year, the devil is not so scary anymore and far preferable to the deep blue sea.
Prognostic Testing
Over the last 10 years or so, we have learned a great deal about what makes CLL tick, what to look for by way of prognostic indicators. Most experts agree that top four prognostic indicators are:
- IgVH gene mutation status
- FISH test (chromosomal abnormalities)
- CD38 level on CLL cells (Flow cytometry)
- ZAP70 level on CLL cells
If you are not familiar with these prognostic indicators and how they influence your “Risk Bucket”, I strongly urge you to read an article “What type of CLL do you have” on the Newly Diagnosed page.
The Mayo study found that prognostic test results were useful in predicting the time to first treatment in all age groups. But they had less success in predicting overall survival – especially in older patients, those over 75 at the time of diagnosis. By the time patients are 75 or older, other medical issues can become more important, their CLL may no longer be in charge of driving the bus as it were.
Of the lot, IgVH gene mutation status was the single best predictor of overall survival for patients younger than 75 at diagnosis. This is also the test that local oncologists seem to balk at prescribing, possibly because it is harder to understand and interpret. If your guy does not “get it”, you have two choices. Go to an expert center where they do get it. Or, learn as much as you can about the test, arm yourself with reputable journal articles on the subject and try to convince your oncologist why getting this test done is important.
Moral of the story: prognostic testing is a lot more important in younger patients. Money and effort spent in getting prognostic tests done are likely to pay off in giving them a better awareness of what they are likely to see coming at them down the road. They have a more complex game plan to implement and any heads-up they can get will be invaluable.
Israeli Study
Below is a very recent paper looking at some of the same issues. This study comes to us from Israel. It is a much smaller study than the Mayo report we discussed above, only 87 patients participated in it.
This paper also documents the larger penalty of loss of life expectancy paid by younger patients. Unlike the Mayo study, this paper suggests that younger patients are likely to have a somewhat more aggressive form of CLL, compared to patients who are older at diagnosis. I cannot swear to its statistical significance, but my own strictly anecdotal database supports this. Over the last seven plus years I have seen many more young patients with more aggressive CLL. Very few of these young guys seem to have classic “smoldering” CLL variety. On the other hand, I have dozens of older patients in my database that have lived 15 or more years without needing any therapy at all for their CLL!
Int J Clin Pract. 2009 Nov;63(11):1601-3.
Evaluating the impact of age and disease on survival of chronic lymphocytic leukaemia patients by a new method.
Klepfish A, Rachmilewitz EA, Sarid M, Schattner A.
Blood Bank and Hematology Institute, Wolfson Medical Center, Holon, Israel. amiMD@clalit.org.il
BACKGROUND: The impact of chronic lymphocytic leukaemia (CLL) on survival may be different in younger patients, but this remains controversial. OBJECTIVES: The aim of the study was to examine the effect of age on survival in CLL using an original method. METHODS: Clinical, laboratory and survival data of 87 CLL patients treated in our institute were analyzed. The survival of patients in different age groups was determined and compared, as related to the expected survival of age- and gender-matched general population obtained from national statistical data. RESULTS: The mean age in the younger (< or = 65 years, n = 37) and older (> 65 years) age groups was 56 and 74 years (p < 0.001). The younger group had more unfavourable presentation, with advanced stage (Rai 2-4) in 46% vs. 16% (p = 0.002), and diffuse involvement of bone marrow in 60% vs. 18% (p = 0.03), compared with the older group, and were more likely to require treatment (p = 0.02). The Kaplan-Meyer curve showed a more favourable survival for the younger group. However, the loss of expected survival exposed a reversed pattern: while the older patients lost only 13%, the survival loss in the younger patients was 44% (p < 0.001). CONCLUSIONS: Chronic lymphocytic leukaemia had a more unfavourable presentation and a more severe clinical course in the younger patients. Our method of evaluating the negative impact of disease on expected survival reveals that their survival also is significantly more affected than that of older patients. We suggest calculating the loss of expected survival as a new criterion for assessing disease impact.
PMID: 19832816
All of us are unique individuals with our own very personal take on things as important as life and death. No amount of statistical data is able to describe our individual zest for life, our willingness to fight one more battle today so that we can see the next tomorrow. Cultural differences are often over looked by physicians. Modern western culture tends to favor ever more intervention, doing anything and everything to prolong life. Not so in some Eastern cultures where fatalism plays a big role in accepting death more readily. Perhaps having fewer healthcare dollars to spend has something to do with it!? What say you?
43 comments on "Does Age at Diagnosis Make a Difference?"
As a recently diagnosed 50 year old, this article has been excellent in answering some issues that seem easier to avoid for our busy NHS Doctors here in the UK.
Thank you so much for the thought-provocation. Just on 4th of 6 part “holding” regime now. It has been difficult to know what to tell my 3 kids about the future. As always, the truth is best. This information helps me a lot o understand that truth.
Best wishes to all.
Chaya
Thankyou for highlighting this interesting article.
Chaya,
Thanks for this very good article, I feel better reading what you have to say as this confirms what I have thought myself over the last few years, and I am only a non scientific patient.
It took me almost 3 years to get some of my test done after a great deal or fight, in those days just a FISH and BMB was thought to be enough so why did I want the other test is my marrow was failing?, was the argument taken by the haematologists I saw at the time.
Ironically after I had all the test done going my own way on returning a couple of years later to my old haematologist I was recommended for a SCT, that would have never happened 5 years earlier.
As I am already 64 going for 65, I assume my life expectancy will not be like someone at 44 having a SCT, but I will be content enjoying a good quality of life for whatever time is left.
Thanks you again for your tremendous dedication to all of us.
All the best for the New Year.
Regards
Chonette (UK)
My husband “looked good” even having bulky disease,odd eye symptoms, bone marrow involvement.He had executive physicals every year; groin nodes were diagnosed as hernias. At 56, the disease was picked up on an MRI for a shoulder injury.Treatment was started a year after diagnosis. Two years into the diagnosis he was chemo resistant,transformed to immunoblastic lymphoma,died 3 months post transplant from an embolus but also miserable symptoms from GVHD,lymphedema and still those bulky nodes. I often contemplate if we would have been better off with early treatment. We walked away from the small town oncologist who wanted to start right away and went to a University where their protocols were watch and wait.
Regards,
Sandy (midwest US)
Chaya,
Thanks for bringing this important study to our attention and interpreting for us. It is very sobering. I sure do hope they cont. to improve results of SCT’s. I was diagnosed at age 51 and 4 yrs later I am not progressing. I celebrate small blessing but realize I have tx on the horizon and possibly a SCT.
Thanks for continuing your work for us.
Chris
Adks NY
Clearly there needs to be a middle ground between watchful waiting and immediate chemo. I’m still in Stage 0 after three years post-Dx. At this point, with all I’ve read about the body’s becoming resistant to first this treatment, then that treatment…I’d rather wait as long as possible until a treatment is discovered which actully cures.
Eve (Northeast US)
Chaya,
Thanks for this very helpful article, I copied this to present to my local Dr. I am one of the lucky few at 39 years old have been diagnosed. I am in a WW holding pattern right now so this will at least give me time to hopefully “get my ducks in a row”. Thank you for all your informative work it will be a big help in my future fight.
Aric (NJ)
Chaya,
Thanks so much for all the information you provide. My husband was 44 when he was diagnosed with CLL. Unfortunately, we didn’t know about specialists at the time and just trusted our local oncologist until 2005 when things really started going bad. Then I found the CLL List and found out about Dr. Byrd, so we went to him. Turns out Mike was 11q, unmutated and his bone marrow was 90% infiltrated. A SCT was recommended, but first they would have to try to get some of the CLL out of his marrow. They’ve never been able to get him well enough for the SCT and it’s off the table now. If only we’d known early on about these things.
Cindy
Lafayette, IN
Thank you Chaya.
Well, here is one of the few times in my life I wish I was 75 instead of 34. I’m stage 0/watch and wait: flow cytometry reveals bright CD 19, CD 5 and negative for CD 38 (just to mention some of the markers), FISH test reveals 13q14.3 deletion and trisomy 18 expression. (Anyone else have trisomy 18 expression?) IGVH test came back unable to detect a monoclonal B cell population and so the test will be rerun at a later time.
Once a month I make my own peripheral blood smear and look at it.
cwarren154
Cindy, it is stories like yours that break my heart. They are also the driver behind my conitnuing this crusade well past the death of my own husband. He too had 11q deletion, aggressive CLL, he too died way too young. If having this information out there helps just one patient make better choices, then it is worth it to me.
All the best to you and your husband.
As always you arm us with information that alas, protects us somewhat against our doctors. I tried to set up an IGHV test in the UK last fall, but even for a $1000 consultant’s fee, could not get a guarantee of the test, so I cancelled. Are there any places in New York State where the test can be done at a reasonable cost? My FISH was okay, but it was like pulling teeth to get it.
Patrick
Perhaps some of our UK members would chime in on how best to get this test done.
I know Quest Diagnostics does the IgVH gene mutation status test and the whole prognostic package including IgVH is less than $1000, back a couple of years ago when we negotiated a package deal with them for our patients. You would still need a physician to write the prescription for you.
I appreciate Chaya’s wisdom in pointing out that “cultural difference are often overlooked by physicians.”
Spiritual differences are also overlooked….and not only by physicians.
Please allow me to offer quotations from two genuinely compassionate physicians, both specialists in geriatric medicine and one a clergyman, who reflect on decisions that many of us make – some sooner than others.
(1) “To say that anyone chooses to die is, in most situations, a misstatement of the facts. But, medical advances have created at least the facade of choice. It appears as if death has made a counter-offer and that the responsibility is now ours.”
(2) “Given even a minimal belief in what we Christians say we believe about Christ rising from the dead, it strikes me that we do many crazy things to people who are facing the end of life. If we are maintaining an ongoing, aggressive, burdensome therapy in a person who is clearly dying naturally, that doesn’t make sense. There can almost be a delusional fixation on a cure that doesn’t exist.”
I agree with Chaya that in the end, whether to aggressively searach for a cure or to run out the clock is an intensely personal choice, influenced by each person’s particular circumstances (age, whether one has dependents, prognosis, etc). Either choice CAN be wrought with hope and optimism; therefore, letting go is not necessarily fatalistic.
Manhattan:
Thanks for sharing these comments. I cannot agree more with the sentimente echoed in each of them.
Each of us faces death in our own unique way. For me, when it is finally time to bid goodbye, it will be almost a sense of relief. My kids are grown up, I have completed my family responsibilities, I think I have been a socially responsible person and fulfilled my “Dharma” to the best of my ability. My house is in order – well, not literally :)For me, the woods are indeed lovely, dark and deep. There are few promises left to keep. When it is time, it will be good to sleep.
I wonder how age at “diagnosis” relates to age when the patient actually develops the mutation.
I was diagnosed at 59 (I’m 64, still on W&W). But at least 5 years before that I was told to stop giving blood because I had signs of type C hepatitis, and also another blood test showed signs of HIV infection. In both cases my doctor did additional tests and said that I did not have these conditions.
Incidentally, this is one reason I’m against universal HIV screening. To take people with no risk factors and say, “You may have HIV” and then,”no, it’s only cancer,” does not seem a useful or humane plan.
I’m perplexed about the lgVH mutation test. I’m a 54-year old man, four years diagnosed, whose CLL is slowly progressing but by no means ‘smoldering’ and as yet to have treatment. I go to one of the foremost CLL docs in the U.S. in Washington, who is up on all the latest research and has had me do flow, FISH and ZAP70 testing a number of times (ZAP70 negative and FISH inconclusive with no abnormalities found). Yet for all his savvy, he has never mentioned an lgVH test. Is it so hard to do? To interpret? So expensive? Why is that test so rarely done? Tho based on your suggestion, I’m going to push for it at my next checkup, at least once, even if my insurance will only cover it in part. As always, thank you so much, Chaya!!
1. History. At diagnosis in 1998 I was at the opposite end of current statistics: Female,Urban, 42 yrs old. One year later I left the corporate world, without having had time to build a sufficient nest egg. Removing the stress of a career by far offset the small worries of making a living so as not to touch savings and investments. In the first few years I relied on Granny Barb’s site for info, and gradually decided that I did not want any chemo drugs. Everything seemed so primitive and lethal- I understood that people died of treatments not of leukemia. I lived in semi-denial of W&W until I stumbled upon CLL Topics. By then I had some prognostic tests done, but it was only the year of treatment (2008) that I had zap70 and IgVH (both good).
2. Cultural Differences. I agree wholeheartedly about differences. In Brazil (and most of Latina America) physicians are not hand cuffed by a choking insurance business, nor by a litigious society as in the US. They are able to practice a more holistic approach to medicine, incorporating complementary therapies, family customs and beliefs, and a strong psychological component; the patient is the center of the treatment and not vice-versa.
This leads to intervention probably later than in the US (my first oncologist in Atlanta said I would probably need treatment within 5 years — I left his practice right then.)
3. Your Database. If you’d like to build a database, would you be interested in setting up a questionnaire for us to fill out?
Chaya
I was diagnosed with CLL in 1994, 61 years old. On W&W for 8 years. After a colon resection, 1999, for colon cancer, I had very large abd. nodes and treatment in 2002.
Not exactly flash-forward to 2010 but at 76 years I am on treatment with rituxan/bendamustine. Some days can be rough but I’m a tough old broad and responding well.
After 15 years with this complex disease, it is a big part of my life.It is not going away. I live with it and it’s ups and downs.
I am so thankful for your updates and valuable information.
Many Blessings,
Rita
Thanks Chaya. Very useful and interesting article. As a young CLL patient (now 45, diagnosed at 40) the article answered a lot of questions and confirmed my conviction that a SCT, despite the risks, should be the treatment for me in the future. The question that comes to mind is how long to wait? The article propels me to want to get a second opinion to answer this question. I have a good hematologist at a major medical institution, but I have long felt that another take on my disease would be helpful in deciding when to act. Thanks for the inspiration to action.
Thanks also to Manhattan. Thought provoking indeed.
Jenny
Thanks Chaya. I always find research like the Mayo Clinic study kind of a no brainer. OF COURSE, a 30 year old is less likely to outlive a chronic, sometimes life threatening disease than an 80 year old. Most people would assume this. The details are never the less interesting and I appreciate the article.
As to the second article suggesting that younger people more often present with more aggessive disease. I would think that one factor could be the fact that agressive disease is diagnosed earlier. How many 30 and 40 year olds are getting flow cytometry performed for a slightly raised lymph count. My doctor just ingnored it. My 80 year old mother’s doctor immediately had a flow performed when her lymphocytes were 5.1. I am not saying that young people don’t , as a group, have more agressive disease, I’m just wondering if there might be some other reasons for it.
Be well,
Debbie
Thanks, Chaya, for starting the new year with this interesting article. My wish for 2010 is that more scientists and doctors will take CLL seriously and consider it ‘worthy’ of further study. I think there is still a climate of denial in some of the medical profession. I was diagnosed in 2004, during preparations for a hip operation. I was not told about the CLL until several weeks after the operation, when I was sent to a hematologist to hear the news. While still reeling from the shock, I went for a post-op checkup with my very senior and highly respected orthopedic surgeon. He bawled me out for saying I had leukemia, and insisted it was unlikely to have any impact on my life, or health. If we are to make more headway, we will need to get more information out there, as Chaya has been valiantly doing. This is a two-way process — educated patients can contribute to the education of doctors, and medical researchers.
Chaya,
I want to wish you and your family a Happy and Healthy New Year! I have been diagnosed with CLL almost 10 years ago.I am 69 years old. I have had various treatments since 2005 including Fludarabin, Rituxan and Campath. I have been in remission for the past 9 months,as well as other periods between treatments, however, my lymph nodes are now enlarged enough that I will require new treatment. Have studies been made of which treatments are best suited to reduce the lymph nodes enlargment? What are your thoughts of the recently approved Treanda for CLL/Lymphoma patients? I really would appreciate a response from you. I am sure that there might be other patients with similar prognosis. Thank you!
Ron Isaacson, NJ
Chaya,
As a male diagnosed with CLL early, I was interested that you may have seen more aggressive forms in those diagnosed at younger ages since it bears directly on my prognosis. Blood testing at 39 indicated early CLL (lymphoproliferative disorder it was called) and three years later CBC, bone marrow, and CT scan confirmed B cell CLL; at the time I was told the median time of survival for me was 8 years. This seemed similar to the CLL my father had, which was diagnosed at age 55 and proved fatal at 74, for my case slowly progressed in total white blood cells (lymphocytes) and then remained pretty stable for a number of years, never requiring any treatment.
At age 56 I retired and transitioned into a healthier, low stress lifestyle and over the last several years my total white count improved from a high of over 60,000 to 7200 this year, though still with 57% lymphocytes. My hematologist said I might have a form of CLL that does not progress. We only did more sophisticated tests back in 1990 so I have no IgVH, FISH, CD38, or ZAP70 results and my doctor couldn’t recommend any of these tests as the results would not alter the watch and wait regimen. I’m wondering whether I am just lucky with the type of CLL I have or whether I have actually done something to improve the disease. Discussing this idea, I was told that some improvements in CLL have been seen with exercising but not with dietary changes. In my research I don’t recall any claims of improvement during watch and wait and I’m not about to say the disease won’t progress later. Have you heard of any claims like mine?
Many thanks for CLL Updates,
Dan (central PA)
ike4573
Refractory patients, especially those with bulky lymph nodes, are the segment of our patient population that is under-served. “Life after FCR” is grim reading; if you have not read this article already, I suggest you do to get a clear view of the lay of the land. Among the more promising salvage therapies are Revlimid combinations. My next article is going to be on a couple of very intriguing clinical trials using this immune modulator. These may prove to be very valuable options for poor prognosis patients.
Another option is flavopiridol. This drug is not yet commercial, you can only get it as part of a clinical trial at Ohio State (Dr. John Byrd). By no means a walk in the park, but flavopiridol has been successful in treating some pretty aggressive cases.
Some researchers think bendamustine (Treanda) may work even in patients who have relapsed after F, C, R and Campath in various combinations. I would put more money on a combination of bendamustine + ofatumumab (Humax-CD20). This too is a clinical trial in progress. Think of bendamustine + ofatumumab as a different version of FCR, bendamustine substituting for F+C and ofatumumab substituting for Rituxan. It just might be sufficiently different that even patients who have relasped after FCR might get another chance to respond.
Some physicians believe in treating bulky lymph nodes as if it is now a lymphoma and not leukemia. RCHOP is a popular option. But beware, this combination has significant cardiac toxicity. There are other lymphoma combinations as well, ICE, RICE etc.
MDAnderson uses combinations such as OFAR and CFAR to treat refractory patients. The former combo uses oxyplatin, a solid cancer chemotherapy drug often used in breast cancer and the like. The later is a mixture of FCR + Campath. Please read our recent reviews of addition of Campath to strong chemotherapy regimens such as FC. You need to be carefully monitored when undergoing Campath + other immune suppressing chemo regimens.
Hope this helps you and others in a similar boat.
Chaya,
Thank you so much for your informative reporting. I have gained much more understanding of this disease from your articles than I have from my doctors. It seems that some of them are not aware of the different types of CLL and I have heard nothing until now about age of diagnosis and aggression of the disease. I’m 56, diagnosed 6 months ago. I have been told I’m ZAP70 positive, 11q,and negative for the mutation. I’ve decided to check out a “for profit” treatment center. The teaching hospital I’ve been to twice is still insisting I’m not eligible for a clinical trial and should just W/W.
Please keep up the good work.
Gratefully,
Culture is important, but in some cultures you treat yourself without a doctor when you can, and hence the horrible resistant TB etc.
Thank you, Chaya, for another interesting article. Although I was not diagnosed until my early 50s, I think the signs may have been there much earlier. Blood tests for menopausal hormone levels in my early 40s showed white blood cells constantly ‘somewhat elevated’ outside the normal range but were dismissed as unimportant. I think there may be lot of undiagnosed CLL out there. As I am still ‘watching and waiting’ at 55(and pushing back the tide a little with green tea supplements), would it have been useful to know earlier? My answer is that I would always prefer to know. To be informed is to be able to make better choices and I might not have pushed my body so hard in the intervening years.
CLL Topics continues to be my best source of information and has a wonderful balance between hard evidence and human empathy. Thank you again.
Julia, Brisbane, Australia
It’s clear from the comments here as well as comments to many of Chaya’s other wonderful updates that the quality of oncology varies dramatically from local generalists to CLL treatment centers like the Mayo Clinic. I am one of the 2,487 patients noted in the study as cared for at Mayo (diagnosed in 2006, still W/W). Switching from a local oncologist to a Mayo expert has proven to be an excellent move. I have had the benefit of participating in the clinical trial of “green tea” therapy, and have the utmost of confidence that the medical advice I receive is state of the art. I’m hopeful that there will be more opportunities for clinical trials that might lead to a cure short of a stem cell transplant.
Thank you Thank you. All I have been doing since diagnosis this summer is read and read more CLL Topics. I am 44, female and 17p deleted. Thanks to you Chaya and all the information I feel like I know what is ahead (transplant) and I am ready, but I also know that it is important to listen to my body and what is going on with me and not look too closely at all the “information” out there. I will remain w&w until the time is right and in the meantime read lots and trust my dr’s to know when the time is right.
Laura, Austin, TX
Hello. I was diagnosed at age 28. Three years later I received RF treatement and have been on W&W since. I’m now 36, and my wife and I recently welcomed our son into the world, so this article is timely as can be for me.
The prospect of a transplant and the difficulty in choosing the proper timing has weighed on my mind for the last eight years. I plan to do everything in my power to ensure that my son grows up with his father by his side.
The better outlook for mini-allos makes the idea of tranplant more and more appealing to me. In addition, having a friend who recently went through the process has taken away some of the mystery. At the same time, I’m well aware of the risks.
This is such a complicated life puzzle we’ve been thrown — but in the end I think the best option for us younger folks is to take advantage of our youth and fight as hard as we physically can. Be well, all!
Dear Chaya,
I definitely owe you and PC a big hug and a “Thank You”! I’m at +90 days post SCT today, have a CR, and doing very well.
I was diagnosed with CLL in Oct, 2006 at age 56. I found CLLTOPICS soon after that and started reading up on everything you had on CLL despite my local oncologist decribing CLL as the good cancer and that I might not ever have to be treated! In 2007, I watched my CBC’s double consistently every 7 months. Due to your infomation, I knew I was going to have to be treated soon and suddenly a SCT was on the horizon. I made an appointment with Dr. Wierda with MD Anderson Leukemia Department and Dr. Khouri with MD Anderson SCT Department at the end of 2007. After consulation with them, I chose to be treated with FCR with Dr. Wierda and began a donor search with Dr. Khouri in SCT as my backup plan if FCR didn’t work. In 2008, I did the 6 rounds of FCR while continuing to see Drs. Wierda and Khouri and determined my sister was a perfect match before the first round of FCR. I also kept reading on FCR sucesses and other treatments after FCR. It quickly became apparent that if I wasn’t in the 70% that FCR gave a CR to, I wasn’t going to live long with continued treatments. And even if it did get a CR, I was headed for a SCT at some point in the future anyway. Unfortunately, my decision day came in May 2009. The FCR didn’t work that well for me (“normal” Fish, unmutated, ZAP 70+, CD 38-) and only gave me PR. Nine months after the FCR, my CLL was startng to progress in my bone marrow again. Per Dr. Khouri’s recommendatons and confirmed by all the data you presented, I chose to proceed with the SCT. I did followup treatments of HDMP + Rituxan to lower the CLL and started the heavy chemo pre-SCT treatment in late Sept, 2009. My transplant was Oct 5, 2009. I’m in Houston about to return to NM at +90 days now and doing well with a CR. It still too early to say I’ve beaten this disease, but no matter what happens in the future, I’m convinced this was the correct decision path for me. I now have a CR and even if the CLL returns, I’ve bought more time and even better, I have a good chance it will never return! It was the informaion and inspiration that you and PC provided that me make the right for me. PC will forever be in our hearts and my hero.
Hugs! And a BIG THANK YOU! Paul Plunkett, Albuquerque, NM
Chaya, Keep on keeping on!
I have a question for “cdaiii dan”. You noted your WBC has dropped over a period of time from 60k to 7k. That is significant to say the least. You did not indicate any treatment. Besides the exercise and less stress can you think of anything else you may have changed or instituted in your life over the past few years? Just curious.
Jim
DavidN,
My husband, Kelly, and I live in a fairly rural area in the Appalachian Mountains in Southwest Virginia. Among others, I requested IgIv testing for Kelly, and his oncologist was happy to oblige. I wish you the same.
Wow! Such wonderful comments from everyone. I am interested in the SCT that pvplumk1 had. Would love to hear the full story of what he went through. Chaya, I can’t thank you and your family enough for providing this information to us. I hope this is a great new year for all of us with new advancements and a cure soon.
The Mayo Clinic report referenced above seems to indicate that the prognostic tests indicate the time to treatment. Is there any information that ties specific indicators to the amount of time to treatment?
zardoz:
This ASH abstract just says all of the prognostic indicators are of value in determining time to first treatment.
However, I exchanged emails with the lead author and he tells me the full length article has been accepted for publication and it has a lot more details than the ASH abstract. I will get a copy as soon as it is public, and I will be sure to bring it to the attention of our members.
Thanks, Chaya, for this informative article. Your updates are my best source of information.
A devoted reader,
Monique
Mocha, I tried to document as much as I could in my CaringBridge journal. I entered something most days but it gets pretty boring after about day +30. Here’s the link. You’ll have to read over the typo’s as I don’t like to proof read and still have a little chemo brain and maybe always did.
http://www.caringbridge.org/visit/paulplunkett
jandg Jim,
The major change that I associate with my drop in lymphocytes is dietary, moving away from the western diet to one that emphasizes variety and moderation. My diet evolved to lower blood pressure and cholesterol, to unload without dieting the extra pounds I’d carried for decades, and to get me into the best possible shape to deal with looming age-related health issues. It never occurred to me that diet and lifestyle changes could improve my CLL, and I still have read of no such claims. There’s no doubt I’m much healthier for the change in eating habits and believing this has helped my CLL aids me in avoiding a relapse into unhealthy eating. You might describe the diet as flexitarian, incorporating parts of vegan, Mediterranean, Japanese, and paleolithic philosophies into a label-defying approach to eating. I do believe that too little variety forces the body to use less than ideal nutrients in its physiological processes, sometimes resulting in disease; this may be what’s behind my drop in lymphocytes or it could be luck or just a glitch in the disease’s progression.
Dan
cdaiii:
I fully agree with your pragmatic take on the value of good nutrition, stress reduction, exercise and healthy life style in general. I do not know of any formal study that links any of these to aggressive control of existing CLL. Exercise “may” have a role – we wrote about it in an earlier article on the Lance Armstrong Effect.
But this much I can say with a clear conscience. Healthy living is a good idea for all of us, it is an EXCELLENT idea for CLL patients. Your life after CLL diagnosis can be a mine field of new risk factors such as increased risk of infections, secondary cancers etc. Just about all the drugs used to treat CLL are tough on your body (liver, kidneys, heart, lungs). Does it not make sense to be in the best possible shape you can be before you take on all these additional risk factors? Getting in shape may not cure your CLL but it will surely make it easier for you to deal with CLL complications.
You mentioned your father had CLL as well. There is a concept called “anticipation” where younger generations in family clusters of CLL have the disease at a younger age and it may be a more aggressive variety. We discussed this in our article “Not the worst day of your life” on http://www.clltopics.org
In the UK the IgVH (and other cytogenetics)test would most likely be done at a University teaching hospital. It is important that the consultant interpreting results is a CLL expert and usually this person is based at a teaching hospital. The district general hospital would be able to request a test, but the samples sent to a specialist centre. It is perfectly acceptable for patients to ask to have these tests done; do not be fobbed off!!
Jaqui
Thanks for very informative article. I have been told on several occasions that I have an ‘old man’s disease’, little comfort when you are female and diagnosed at age 40. Had FCR 3 years ago and currently in remission – now 46 years old. Have not had full diagnostic tests, don’t seem readily available in the UK, but will now push for them at my next consultation.
Keep up the good work – invaluable info. keeping us informed in a language that we can understand!
Jackie
Ihial2
I am in the W&W period in the last 3-4 years. I love Physical activity
( tennis, gym, and golf) and hope very much that I can carry on for more years to come (I am now 73). My question when do I have to insist on doing the tests – lgVH, FISH etc. I intend to get second opinion on
my entire situation and if you can supply me with good questions to ask the doctor I will be grateful. Thanks very much for this most interesting article
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