One of the commonest questions that I am asked is "What questions should I ask when I see my doctor about CLL?"
This is quite a difficult one to answer, because it depends what you are trying to find out. Are you trying to find out whether you doctor is competent to advise you about CLL? Or are you trying to find out about your CLL?
The fact is that some primary care physicians know this about CLL: It is one of the four types of leukemia and it's the one that has the best prognosis. If you've got to have cancer, it's the sort of cancer you would want to have. You might then get referred to an oncologist.
Some oncologists know this about CLL: It is one of the four types of leukemia and it's the one that has the best prognosis. If you've got to have cancer, it's the sort of cancer you would want to have. If you’re lucky you might get referred to a leukemia specialist.
Some leukemia specialists know this about CLL: You're lucky you don't have AML. CLL is the one that has the best prognosis. If you've got to have cancer, it's the sort of cancer you would want to have. If you’re lucky you might get referred to a lymphoma specialist.
Some lymphoma specialists knows this about CLL: You'd better have a lymph node biopsy. If you are really lucky you might get referred to a CLL specialist.
Some CLL specialists know this about CLL: I can cure you with modern treatment. He will greet you in a friendly manner, ask about your family, explain how treatment has improved over the past few years, order a bone marrow biopsy, infuse the first course of FCR and then send you off to be treated by your local oncologist. Your insurance company will receive a barely believable bill.
Now I might be being overly cynical; there are clearly doctors in all these categories that are extremely good, know their stuff and will give you excellent advice. American medicine is among the best in the world: and among the worst. Health care should come with a health warning that says: Market Forces Apply. Caveat Emptor.
The thing about markets is that there are losers and winners, and the winners will win out in the long run. If all you have to lose if you pick a loser is your money, then that might be acceptable, but if you are gambling your life on picking a winner, then you had better pick your doctor very carefully.
So what do you really want to know?
First of all, have you really got CLL? There are two conditions that are easily mistaken for CLL - mantle cell lymphoma and splenic marginal zone lymphoma. The way to distinguish them is by immunophenotyping. So this is a test you need to have. Look for cells that are CD5/CD19/CD23 positive. If they are CD5 negative they are likely to be splenic marginal zone lymphoma, and if they are CD23 negative they are likely to be mantle cell lymphoma. These are not the only differences and it's not as simple as that, but your doctor should be aware of the differential diagnosis and know how to tell the difference. There are other conditions that get confused; you may sometimes be told you have T-cell CLL. There is no such thing. There are three conditions, T-PLL, cutaneous T-cell lymphoma and T-cell LGL leukemia that get called T-CLL. These are all important diagnoses in their own right, but I won't consider them here in this essay; I will write about them later. You may also be told you have B-PLL or sometimes CLL/PLL. again these are important conditions that I have written about earlier this year and I shan't cover here.
The next thing you need to know is what stage you are. Most people in the world use Rai staging, named after Kanti Rai from Long Island. Stage 0 means you just have a lymphocytosis. Stage 1 means that you also have lymph nodes that can be felt. Stage 2 means the doctor can feel your spleen. Stage 3 means you are anemic and stage 4 means your platelet count is reduced. Notice that I said 'feel' and 'felt'. You haven't had a CT scan yet. Rai staging and the conclusions derived from it are based on what the doctor can feel, not what the CT scan shows. Rai staging helps the doctor work out what the doctor should do with you. If you are stage 3 or 4, it is likely that he will recommend treatment, if you are 0, 1 or 2, it is more likely that he will recommend waiting a while.
In fact the National Cancer Institute (NCI) has issued guidelines of when to start treatment. So a good question to ask is, "Do I meet the NCI guidelines for treatment?" If you do not you should be assigned to a watch and wait program. Some people call this watch and worry, but there is a good way of taking the worying out of the waiting and that is to get your prognostic markers done.
If you are stage 0 and your lymphocyte count is less than 30,000 per microlitre the the marker I recommend is CD38. Our experience is that if your are CD38 negative with this condition you almost certainly won't need any treatment and you will never die from the condition. If you have almost any other type of CLL I strongly recommmend that you have your IgVH genes estimated. These will tell you whether you fall into a good or bad category of CLL and give you an estimate of what the future holds. The other prognostic test that I strongly recommend is FISH for deletions of 17p and 11q. I will be writing about these in a future blog, but briefly these chromosome abnormalities are present in patients who either don't respondto standard types of treatment, or if they do, will relapse withing a short period of time. Obviously, these tests will help you decide on what type of treatment you should have.
Finally before you start treatment I would recommend that you read my articles on What is the aim of treatment here and here and here.