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The First Steps

A CLL Primer

Updated: March 23, 2024

by Chaya Venkat

An Introduction and Guide for the Newly Diagnosed Patient

Useful Tools for Patients:
Your Charts;
Reference;
Textbooks;
Search Tools;
Patient Advocacy for Times of Rapid Change.

Here is how it usually happens, a sneak attack you were not expecting that leaves you confused and more than a little scared. You have just been diagnosed with this nasty stuff called CLL, probably following a routine blood test. If you are like most people you have never heard of this cancer. Chronic lymphocytic leukemia: what the heck is that? Doctor mentions the “Big C” and your brain blanks out the rest of the consultation. How can you possibly have cancer when you don’t feel in the least bit sick?

And the doctor says you are not to do anything about it, just wait. Wait for what? Wait for how long? That can’t be right, can it? Aren’t you supposed to attack cancers right away with all guns blazing? He says you are in a “Watch & Wait” mode. Watching for what, for heaven’s sake?

After you get over the shock of it all you begin checking out internet sites looking for information and support. Hopefully you found our site sooner rather than later.

Welcome

If you are a CLL patient or you are the caregiver for someone who has CLL, welcome to our patient community. This is a strictly volunteer effort. We have no ties to the medical or pharmaceutical establishments. Our only agenda is educating our members so that they can take better care of themselves and patient advocacy to try and improve the therapy options available to all CLL patients.

Access to our site is free of charge. We are not interested in selling you anything. We cannot give you medical advice since that is not our job and we are not medical doctors. But we think we can make you a savvier patient able to make smarter choices in the months and years ahead. Most CLL experts – and we work with all of them - recommend our website to their patients.

We try to stay pretty much on-topic in our discussions. The bias is definitely towards evidence based medicine, patient empowerment, the latest developments, new clinical trials and the critical evaluation of the results of such trials. We also tend to be pretty hard-nosed about the miracle cures, one-of-a-kind anecdotal stories that you cannot hang your hat on, TV infomercials that claim to cure everything except the condition of your wallet. We preach a certain amount of skepticism is necessary to judge the real value of the latest miracle of modern science – or the herbal remedy recommended by your brother-in-law’s best buddy Bruce. As you can see, we have a bit of an attitude.

CLL is a non-discriminatory cancer. While it is generally true that patients tend to be in their 50’s or 60’s when diagnosed with this disease, and more men than women get it, I know of plenty of young women and men who got in their mid-30’s. Cancer diagnosis is a scary thing at any age, but somehow it is all the more poignant when the patient is young, often a parent with young children, with jobs to go to and mortgages to pay.

CLL is a confusing disease. On the one hand it is an “indolent” leukemia, with an average life expectancy of 7 or more years. That may not seem like much to you, especially if you are one of the younger patients with this “old man’s disease”. But just compare that to someone diagnosed with, say, pancreatic cancer – where the clock ticks much faster and life expectancy is measured in months and you get why CLL is often called a “good” cancer.

But along with the “indolent” description goes the next not-so-comforting adjective: CLL is an incurable cancer. Bummer! You don’t have to worry about dying right away, but chances are that you will also have to learn to live with this disease, there are no slam-dunk miracle cures out there that will get this monkey off your back, with no fuss and no muss.

Scared? We can understand that. Hopeless? Not on your life!

We want you to know that real and important breakthroughs have been made in our understanding of CLL in the last decade. This has revolutionized how we treat patients, extend their lives and yes, even CURE this incurable cancer. Life expectancy is gradually increasing, patients live with better quality of life and modern stem cell transplants that offer the prospect of a full cure are coming up the curve awfully fast. For the first time we face a future where it is possible to cure this hitherto incurable cancer. How great is that!?

A whole lot depends on you, what you are willing to learn and do to take care of yourself. You have CLL. Like it or not you own it and no one has more skin in the game than you and your family. We hope you will treat our website as an “Owner’s Manual”. We specialize in translating medical jargon into plain English. We do the hard work of reading zillions of articles in professional oncology journals so that we can bring you stuff you need to know and can use.

No, it is not going to be a cake walk but if you are willing to stick with it you will come up the learning curve. Don’t worry if you are not 100% clear about all this stuff right away. No one expects you to understand it right out of the gate, not unless you are a trained hematologist. But I promise you it will become clearer over time, if you are willing to stick with it and do your share of heavy lifting. And time is something that CLL affords us, compared to many other types of cancers. Dealing with CLL is not a make-or-break sprint. It is instead a long duration marathon. You need smarts and strategy to win marathons. We are here to help you with that.

Below is a quick "cheat sheet" of stuff you probably need to know right away, as you begin to get your arms around this crisis in your health. As time goes on, you will learn more details and feel more confident in the choices you make.

Step One: You Need to Get Yourself Reliable Medical Advice.

The science of immunology and our understanding of CLL are changing so fast that many of the less-informed oncologists are getting left behind in the dust. And that is a huge problem for patients. Not everyone lives within a stone’s throw of a world class cancer center. Most of our patients are diagnosed and treated by local oncologists who deal with a variety of cancer patients. Blood cancers are fewer in number than solid cancers such as breast cancer, prostate cancer, lung cancer etc. And there are a dozen or more different types of blood cancers, CLL is one among many.

How many CLL patients does your local oncologist see in an average year? Possibly as few as two or three CLL patients, if it is a small practice. That does not add up to a lot of experience. Is your guy up to speed with all the latest info, does he attend professional conferences and take CME (Continuing Medical Education) courses so that he is familiar with the latest best practices developed at expert centers? Here is a scary thought: what your oncologist does not know can kill you a lot sooner than otherwise.

If you learn nothing from this site other than the need for “Buyer Beware” when it comes to healthcare, consider yourself ahead of the curve. I am absolutely convinced that active participation and understanding of this disease and all of the available options open to you can make the difference between good health and quality of life for many years, versus poor choices that may cost you dearly. This is what makes patient education and empowerment so important for CLL patients.

Step Two: It Is Absolutely Essential That You Get an Accurate Diagnosis.

Chronic Lymphocytic Leukemia (CLL) is a blood cancer characterized by an uncontrolled accumulation of large numbers of malfunctioning white blood cells (specifically, B-cells) in your blood, bone marrow, lymph nodes and other organs such as the spleen and liver. Unlike pediatric leukemias, CLL is almost exclusively diagnosed in adult patients, often elderly patients.

There are other blood cancers, kissing cousins of CLL so to speak, that also have the same basic pattern — for example Mantle Cell Lymphoma (MCL), Pro-Lymphocytic Leukemia (PLL) and various varieties of Non-Hodgkin's Lymphoma (NHL). Some of these are much more aggressive cancers and may need more immediate attention, others less so.

On a personal note, when my husband P.C. was first diagnosed after a routine blood test, the local oncologist told us he had advanced (stage 4, the highest stage) NHL with a life expectancy of about two years if we were lucky and that as we head out the door we should make an appointment to start chemotherapy right away. As it turned out, the oncologist was wrong on all counts. What he had was CLL, and an early stage of it at that. He lived for seven wonderful years with absolutely no loss of quality of life.  Given the swift kick in the butt of a CLL diagnosis he got into better shape than he ever had been in and we learned to value each day that came by. He was never hospitalized during those seven years, never in pain, never had to cut back on his hiking or running.  He was our webmaster, editor and the driving force behind this crusade. When he passed away in the summer of 2024, it was due to an uncontrolled infection following a stem cell transplant.  He came very close to a full CURE, we almost succeeded in getting this monkey off his back once and for all. It was not to be and I will miss him for the rest of my life.

Would he have had those seven years if we had meekly accepted the wrong diagnosis and therefore went along with the wrong game plan?  I do not think so. I cannot overemphasize this point: the most important step is to get a rock-solid diagnosis of your situation. Get a second or third opinion, as many as it takes until you are sure the job is done right and you know the exact name of your enemy. How can you defeat something you do not understand?

Step Three: CLL Comes in Many Flavors — It Is Important to Know Your Personal Risk Bucket.

Recent research has confirmed that not all CLL patients have the exact same type of disease. Some have what has come to be labeled as "smoldering CLL". These are patients who clearly have CLL, but it is of a variety that will not do much of anything for a long time. For these people, the best choice may be no therapy at all. Most patients are advised to follow Watch & Wait (“W&W”) as the initial game plan. Your oncologist does the watching, while you do the waiting.  Or, as most patients put it, your doctor does the watching off and on when he is thinking of you and you do the worrying — full time. CLL is not an easy disease for you control freaks out there with type A personalities. This disease needs cunning and long term strategy, not aggressive short term tactics.

Almost all CLL experts now agree that it does not pay to treat “smoldering” patients with aggressive therapy if they are not likely to need it in the first place! Other CLL patients, not as lucky, may have a more aggressive form of CLL and may need to be monitored more closely and may receive therapy sooner. Doing too little when faced with an aggressive situation could be as dangerous as doing too much in a smoldering case.

The point to take away from this section is this: after making sure that what you have is indeed CLL, it is important to know what variety of CLL you have. One of the biggest emotional hurdles faced by cancer patients is getting a good reality check on their specific situation. A short decade ago we had no way of telling ahead of time which patient was going to need treatment sooner, which one was going to be lucky and die of plain old age some day in the distant future. Now we have a pretty good handle on modern prognostic indicators that can give you a head’s up on the lay of the land.  The crystal ball is not entirely clear, and there are always exceptions to the rule.  But by and large, it is now possible to get prognosis at diagnosis. In the list of your reading assignments is an article titled What Type of CLL Do You Have? This is probably our most widely read article and it will help you figure your personal risk bucket.

Step Four: Get the Right Diagnostic Tests Done

This is where it gets a little complicated, and there is no way to avoid the jargon. Further down in the reading list for newly diagnosed patients are articles that will help you understand your blood test results.  Stick with us, I promise you all this will become a lot easier if you don’t give up.

The first test ordered is usually a blood test, called a CBC (Complete Blood Count), which looks at the numbers of the various kinds of cells in your blood. In addition to the CBC, the doctor may also order a blood electrolytes test, to make sure your kidneys and liver are functioning well and your electrolytes are in good balance. This is also called a chemistry panel or a CMET (Comprehensive Metabolic Panel). Less frequently, and perhaps only if you are getting frequent infections, an immunoglobulins test may be ordered, to measure the level of these important infection-fighting proteins in your blood.

Reading one of these lab reports is not easy since they are loaded with acronyms that make no sense to the layperson. Over time there will be so many blood tests that the paperwork will accumulate at an alarming clip and your brand new three ring binder inadequate for the job. You might want to look up the section of this website titled Your Charts . The Microsoft Excel workbook named Records.xls provides worksheets containing tables and charts to help track your medical data. Each of the table headings is commented: as you slide your cursor over them, you get a brief description of the term and what it means. You can download and save this spreadsheet on to your own computer and if you wish, modify it to suit your needs. Getting organized will make it easier for you to track and monitor your test results. Most doctors love an organized patient that does not waste their time.

The CBC may tell you and your doctor only that you have an elevated number of white blood cells in you blood, seen as a high "WBC" (White Blood Count), and higher than normal number of lymphocytes, reported as “ALC” (Absolute Lymphocyte Count). This could easily be due to a simple infection. In fact, every time you come down with a bug of some sort, your WBC will ramp up because your body is marshalling its disease fighting armies (of which B-cells are an important part). You may even have slight swelling of the “glands” (lymph nodes) under your chin. But if the WBC and ALC continue to be elevated, the glands continue to be swollen, there is no noticeable infection to blame, then it is time to look more closely at the cause of the elevated WBC and ALC.

I want to repeat this important distinction: increased WBC and ALC by themselves are not a definitive indication of blood cancer. You and your doctor will have to look at finer details to make that determination.

One of the blood tests that is done to make this determination is something called "flow cytometry". This tests looks at the lymphocytes in more detail, specifically B-cells. Cancerous B-cells, whether they are CLL or some other type of blood cancer, have very distinct sets of markers on them, markers that are not present on healthy normal cells, or not present at the same levels, or in the same combinations. These markers are called "cluster designations" or "CDs". There are more than two hundred of these CD markers that have been identified and studied.

One of the things the flow cytometry looks for is the level to which your B-cells have the classic pattern of CD5, CD19 and CD23 markers, since this combination of markers is a seen in all CLL patients. The flow cytometry will also look for other markers that are indicative of some of the other types of B-cell malignancies we mentioned above, in order to rule out these close cousins. The results of the tests for the CD markers are sometimes reported as "dim", "moderate", or "bright". Other labs may use the notation of "1+", "2+" or "3+" for saying the same thing. In most cases, combination of elevated WBC, ALC and flow cytometry results from a competent testing lab are sufficient to confirm CLL diagnosis.

Sometimes the doctor may ask for a CT scan or PET scan, if there is concern that some of the internal lymph nodes that cannot be felt by physical examination have become very swollen or placed such that they may threaten important organs or squeeze shut major blood vessels. These scans can also show if any of the other organs, especially the liver and spleen are presently involved.

CT scans and PET scans are all the rage with some doctors but most often they are not needed for the purpose of diagnosing CLL. I personally do not like getting tests done that I do not need, and I think you might want to ask your doctor to explain to you why these scans are being requested. Besides being expensive (it does not matter who pays for it, you or your insurance company, in the final analysis the only ones who pay for anything and everything are the taxpayers – that means you) some of these tests can expose you to levels of radiation that you may want to avoid if they serve no real purpose in your case. In any case, it is an excellent idea for you and your doctor to get used to the idea of you asking questions first, get the relationship off to a good start where you both participate in the decision making.

In my husband's case, the local oncologist also ordered a lymph node biopsy. This is a minor surgery, but nevertheless surgery that needs general anesthesia, and significant discomfort later as the incision heals - besides being expensive. We were way too naïve and trusting back then and we did not refuse the surgery. The specialist we saw at the expert center later on was aghast that the biopsy was ordered so lightly, since there was absolutely no need for it and nothing was learned from the biopsy sample that could not have been learned more easily from simple blood tests. I guess the moral is this: you need to be pro-active in asking why specific tests are being done, and equally assertive in asking for other tests that may not get done if you stay passive. You are your own best advocate.

In addition to the blood tests, and perhaps scans, another test that is sometimes requested by oncologists is a bone marrow biopsy (BMB). There are two schools of thought on this one. Some CLL experts (a decreasing number over time) swear by this test, since it gives them a clear look at the bone marrow, the most important site of the disease for CLL patients. Other experts feel that blood based tests are now so sophisticated, it is not necessary to go through the more expensive, often more painful biopsy, especially in newly diagnosed patients. More and more CLL experts these days do not require a bone marrow biopsy to diagnose garden variety CLL.

As it turned out, our specialist at expert center we went to felt a bone marrow biopsy early in the diagnosis stage would give a reference point to compare to later on, sort of like getting your first breast mammogram or colonoscopy when you turn 50. If you get the BMB done at one of the expert centers, where they do literally dozens of them every day, the technicians are experts at doing it with little or no discomfort. Really, the description of the process of obtaining a sample of bone marrow sounds a lot worse than it is in reality. If you are going to get a bone marrow biopsy done, it really pays to get the pathology slides examined by someone who knows what he or she is doing.  Why go through all that expense and pain, if the slides are examined by someone without the necessary level of expertise to understand what he is looking at? The results of any of these sophisticated tests are only as good as the intelligence and expertise behind the pair of eyes looking at the pathology slide.

In addition to the flow cytometry report we discussed above, your oncologist may also order some of the prognostic indicators that will give him (and you) a better understanding of your specific situation. You can find a wealth of information on many of these prognostic indicators (CD38, beta-2-microglobulin, IgVH gene mutation status, CD23, FISH, chromosomal aberrations, etc.) on this website. If your oncologist is not the type that is inclined to order these more expensive tests, I suggest you get yourself up to speed quickly on what they mean, so that you have a good set of arguments to present to him or her as to why these tests are both necessary and prudent. Without this prognostic information, and a reasonable understanding of what these prognostic indicators mean, it will be difficult for you to make the right therapy decisions.  CLL comes in so many flavors that I think it is important to get a good view of the lay of the land.

To help you get started on some background reading, you will find a concise overview of the disease in an article titled "Chronic Lymphocytic Leukemia" at Mayo Clinic website. You can also check out Chronic Lymphocytic Leukemia Frequently Asked Questions (maintained by David Thomas) which gives short and sweet answers to many of the questions you may have right now. 

Step Five: What to Do While You Are Waiting

There is no question about it, like most things in life dealing with CLL is a question of hurry up and wait. It drove me crazy. “Watch & Wait” is really tough on take charge type A personalities just itching to engage the enemy. I am not good at twiddling my thumbs when faced with something that threatened my family.

But there are many things you can do, while you are waiting for the umpteenth little detail to fall in place. Think of this as a weather advisory. A storm is brewing.  It is coming your way. With luck, it won't be a real bad one, with luck you will have plenty of time to prepare. But it really is prudent to prepare before the storm gets where you live.

If your body is in good shape, well nourished, well exercised, well rested, unstressed, it is likely to handle both the CLL and the possible therapies that become necessary a whole lot better. Good levels of exercise and nutrition are now even more important. If you smoke, please quit. If you are into sun bathing and tanning salons, it is important for you to know skin cancer is the single biggest secondary cancer in CLL patients and it is a lot more dangerous to boot. It is important to limit your alcohol intake. Keeping your liver in good shape is important since this organ is often under attack by CLL and in any case majority of drugs used to treat CLL are tough on the liver. Be sure to stay up to date on routine stuff like mammograms, pap smears, prostate checks etc.  Just because you have CLL does not mean you get a free get out of jail card for everything else life throws at people.

On this website you will find frequent warnings about snake-oil sales pitches. Anxious cancer patients are a vulnerable lot and there are many nasty folks out there willing to exploit you and make a buck off of your misery. Hang on to your wallet, if something sounds too good to be true, it generally is. When in doubt, raise the question with your doctor, do some digging on your own or ask a knowledgeable expert. Always consider the source. Information from a reputable research institution would clearly have more weight than anecdotal information about miraculous cures from herbal medicines that are never reported in peer reviewed professional journals. Remember that when someone chimes in with an answer on internet chat rooms, the answer is only as good as the source.

On this website there is an extensive list of references and online links that you may find useful, as well as links to many excellent online college-level textbooks that are available to be perused online at your leisure, free of charge, courtesy of the National Center for Biotechnology Information and your tax dollars. Research in the life sciences is advancing at a phenomenal rate, and for those so inclined, reading about it can be a fascinating experience. This is amongst the most exciting fields of scientific adventure in today's world.

Step Six: Use this Website to Educate Yourself

For when you get serious about it, there is an extensive list of major topics discussed on this website on our Contents page. Clicking on a topic heading on this list will take you to a page devoted to that topic - and here you will find introductory blurbs to a number of articles subject, or short discussion pieces. You can read short introductions to some of the more important recent articles on the Home page and use them to link to the individual articles. You can also see a listing of the most recent articles on the website on the Current Articles page. For those who want to know the best ways of getting around the website and discovering all its resources, we recommend Navigation Notes.

Remember, in dealing with CLL your mileage will vary depending upon how well-informed you are. 

Bottom-line: What It All Means

The old paradigm for CLL was that only old people got this disease.  There was no cure for it, so the best thing to do was to keep the patients in "Watch & Wait" mode as long as possible, waiting until the disease gets to the point where the patient is beginning to experience serious "B-symptoms".  The conventional game plan was to treat the patient with drugs that gave short term remissions, made the symptoms easier to bear. No attempt is made at curing the patient — the drugs were considered to be palliative and not much more than that.  And all that was required of the patient was passive “Waiting”.

This approach made a lot a sense just a few years ago, since there really were no drugs that could cure CLL and most available drugs were toxic to other systems in the body. Adverse effects due to older chemotherapy regimens could cause quite serious and sometimes permanent harm. It was often a case of the cure being worse than the disease, so the logic was clear: wait until the last moment to initiate any treatment. The drugs available even a few years ago showed no survival advantage — in other words, patients who took these drugs did not live any longer than those who did not.

The scene is changing now, especially since CLL patients are now being diagnosed at earlier age, possibly because of more widespread use of annual medical exams and routine blood tests. It is not as much of a penalty to a 75 year old patient to be told he has CLL with a median survival expectancy of 7 years. The same message given to a 30-50 year old man/woman packs a bigger punch. "Watch & wait" followed by palliative therapy is no longer good enough, if it ever was.

The good news is that for the first time it is possible to plan for a long and healthy future for many CLL patients, with survival statistics that match "normal" people of the same age and sex. A significant percentage of the patients diagnosed with CLL today will die of plain old age, heart attacks, traffic accidents or whatever, causes that have nothing to do with CLL. Some of this is because of better prognostic tests, giving doctors a better handle on whom to treat, when to treat and with what.

The other side of the coin is that the discovery of monoclonal antibodies (Rituxan, Campath, Arzerra) have revolutionized the treatment options open to CLL patients. In combination with other immune system modulators (Revlimid) or standard chemotherapy drugs, these “smart” and targeted drugs have made it possible to get better responses, deeper remissions and higher percentages of responding patients, all with lower toxicities and better quality of life. For the first time, patients are living longer because of treatment!  Wow, how neat is that! Last but not least, we have come a long way in improved support for the "side-effects" of CLL: better drugs to handle opportunistic infections, better ways of combating disease-related anemia, reduced platelets and other auto-immune diseases, better guidance on how to minimize or avoid secondary cancers.

All in all, the future looks bright and I am betting my money on things improving even more in the years to come. In the meantime, there is a lot you can do to help yourself. It is your job to stay as healthy as you can, learn as much as you can and take charge of your own healthcare the best you can. CLL Topics is honored to help you in this fight.

Chaya Venkat

Founder
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