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(Announcer)
Major funding for Second Opinion is provided by the Blue Cross and Blue Shield Association, an association of independent, locally owned and community based, Blue Cross/Blue Shield plans, committed to better knowledge for healthier lives.
(clock ticking)
(music)
(Dr. Peter Salgo)
Welcome to Second Opinion, where each week we solve a real medical mystery, where we close this file, and half an hour from now, you'll not only know the outcome of this week's case, you'll be better able to take charge of your own healthcare. And by the way, doctors will be able to listen to patients more effectively, too. I'm your host, Dr. Peter Salgo. You've already met our special guests who are joining our primary care physician, Dr. Lou Papa. Lou, good to have you back.
(Dr. Lou Papa)
Thank you, it's good to see you.
(Peter)
No one on this team has seen this case except me, so we're going to get right to work, and we'll roll this one out. Let me tell you a little bit about Sarah. She's 67 years old. She's in your office, Lou. She's complaining of night sweats and a fever. What are you going to do?
(Lou)
Well, that's a wide list of things that can cause them. I'm going to want a little more information, if there's any other symptoms that she has. If she has a cough?
(Peter)
Well, I can tell you that she can't give you much, other than the sweats have been going on for three weeks. She has a thermometer, which surprisingly, to me, measures her temperature in Celsius, because that's how it's in the chart.
(Dr. Lou Papa)
Okay.
(Peter)
38.5 at it's top. She's never had chills. Never had a rash. She's lost four pounds. She's not trying to lose weight. Any other history that you need?
(Lou)
So, I'm assuming, since you don't have it, there's no other symptoms. There's no cough, there's no diarrhea, there's nothing like that.
(Peter)
Not complaining of cough, not complaining of diarrhea.
(Lou)
And there's been no new medications, she hasn't traveled recently?
(Peter)
She went to Europe 18 months ago. Otherwise, she was born and raised in the United States. And since you didn't ask, but I'm sure you'd like to know, doesn't use tobacco, has no allergies. She has a history of hyperlipidemia, high blood pressure. That's it.
(Lou)
Okay. And she doesn't take anything for that, or she does take medication?
(Dr. Peter Salgo)
Not that I can find here in the chart. It's an incomplete chart.
(Lou)
So what's going to be important, is a good, thorough physical examination, with fever, weight loss. That's going to be critical.
(Peter)
All right. I can tell you that, here in the chart, her vital signs are normal in the office. Her doctor notices some cervical lymph node enlargement, more on the left than on the right. They're palpable and they're not matted. Do you want to translate that into human speak?
(Dr. Lou Papa)
Well, I mean. That's concerning. I have somebody who has a fever, a fairly significant fever, weight loss, a loss of appetite and now lymph nodes on her examination. That still is a fairly broad differential. Infection is still possible that some "immune conditions" can be considered malignancies.
(Peter)
Lou's got a long list of differential here. Is anybody concerned about this patient, anymore than Lou is, and what, what diagnoses come to your mind?
(Richard)
So I think we need to know something about the swollen lymph nodes or glands in the neck.
(Peter)
Okay.
(Richard)
We need to know how big they are, how hard they are, what they feel like.
(Peter)
This is it. They're palpable. They're not matted. I can tell you that they're painless. And I don't have an estimation here in the chart of the size that they are.
(Richard)
That all makes a big difference, though, because if these are very small, little pea-sized things, you might be less concerned. If they're several centimeters or several inches wide, and they're hard and firm, you'd feel a lot more concerned.
(Peter)
Let's get some definitions down. I mean, we've thrown this word out, you know, lymph nodes, or masses in the neck. What are lymph nodes? What are they doing in your neck in the first place? And what do they do when they're not getting big and causing trouble?
(Richard)
So lymph nodes are really called glands, in the common vernacular. The way you find them, they're there all the time. They work against infection. They help protect you. But what happens is, when you get a particular condition involving them. For example, if you had a cancer in there, they would be enlarged, and they would be relatively hard, and you would find them as a lump or a bump. You'd feel something on your neck.
(Dr. Peter Salgo)
Joel, you had a similar complaint. You had some lumps in your neck, went to see the doctor. What did your doctor say?
(Joel)
Doctor said it was time to do some tests. Apparently they were large enough and otherwise satisfied his concern, that they went on with a needle biopsy.
(Peter)
So they actually...
(Joel Seligman)
CAT scan, PET scan.
(Peter)
They put a needle in those lumps. And you didn't wait at all. I mean, Sarah did a little bit. You just said, lump, doctor. What prompted you do that?
(Joel)
Not quite.
(Peter)
Tell me about...
(Joel)
I had no symptoms. I was aware of the lump. I called the doctor. I had a regular appointment two weeks later, and then I saw him.
(Peter)
Okay, so two weeks.
(Joel)
Yeah.
(Peter)
Okay, I'll tell you what happened with Sarah. She did wait, as you know, several weeks. She was sent for blood tests by her doctor, and a chest x-ray. The blood tests all came back, it says here in the chart, normal. Her chest x-ray was normal. And they scheduled her for a biopsy of the lymph nodes of the neck. I know you already mentioned this word, the C word, but spell it out for me a little bit. What is this biopsy, and is that what Joel had?
(Richard)
Same kind of thing Joel had. If we're worried about cancer, or sometimes even other things in those lymph nodes, if they're big enough and they're suspicious enough, then we'll have a surgeon make a small incision usually, and try to take out that lymph node. Occasionally, they'll do it by a needle directly through the skin. But either one of those can help get a piece of the tissue for the pathologist to look at.
(Dr. Peter Salgo)
Sarah went ahead. She had a biopsy and the results came back. It says here in the chart, fairly quickly. She heard, "cancer of the glands. There were clusters of medium to large atypical lymphoid cells with cleaved nuclei." Here comes the word salad, right. "CD20 positive, bcl-2 positive, bcl-6 positive, CD10 negative," I'm almost out of breath. "CD23 negative." Help. I'm not...
(Dianne)
I hope Sarah had someone with her when all of this information was shared with her, so that somebody else could be listening.
I would imagine she would have shut down the minute she heard the word, cancer.
(Peter)
Yeah, she heard the word, cancer. I'm pretty sure she didn't hear the rest. I'm not sure the rest of this melange would have made any sense to anybody anyway. Elizabeth? This kind of cancer, specifically lymphoma, is it a rare kind of cancer?
(Elizabeth)
No, it's not particularly rare. It's one of the 10 top most common cancers, and it occurs at about 66,000 people are expected to get lymphoma in the U.S. in 2008, and about 19,000 will die of the disease. But the good news is, there's about a 65% survival rate, five-year survival rate.
(Peter)
Five-year survival.
(Elizabeth)
Yeah, relative survival rate.
(Peter)
Let me just stop there for a minute, because we use this five-year survival as a marker. You survive for five years. We think that's great. Everybody thinks that's great. Does five years mean you're cured?
(Elizabeth)
No. Not for lymphoma, necessarily.
(Peter)
So why do we use five-year survival as a marker for anything?
(Dr. Elizabeth Holley)
Well, it's a good marker. It's a benchmark, whereas if you think about other cancers, like pancreatic cancer, for instance, five-year survival rate is dismal. Very, very low.
(Peter)
Sarah has just been told she has cancer, Karen?
(Karen)
Right.
(Dr. Peter Salgo) Has she heard anything...
(Karen)
Probably....
(Peter)
....at this doctor's office visit, other than, you've got cancer?
(Karen)
Probably not. She needs somebody with her now, to be there, and she needs a paper and pen, so she's writing down what she's hearing, and so she can also write down her questions before she goes to the doctor, so she makes sure, because not only does she forget when she hears the word, cancer, but one of the things that leaves her mind, is all the thoughts that she was having, that she had questions about, before she came in.
(Peter)
Joel, you got this diagnosis, right. You had a lymphoma. You were in the office, I would hope.
(Joel Seligman)
I was in the office, and you don't stop, you've got cancer. I mean what kind of cancer? What stage, what's the prognosis? What's the treatment going to be? There are 260 different kinds of cancer, and how serious it is, is not based upon some aggregate, like non-Hodgkin's lymphoma, it's based upon your specific diagnosis.
(Peter)
Let me ask you a more fundamental question, Richard. Here we have a diagnosis. She has a non-Hodgkin's lymphoma. How do you want to treat her?
(Richard)
That's not a diagnosis. That's a category of cancers.
(Peter)
Okay.
(Richard)
We need more information, because the one thing we have to separate, I like to think of, there are aggressive types, and there are slow growing types.
(Peter)
Okay.
(Richard)
We need to know which one she's in at the very first level. I would like to know whether this is a follicular or a diffuse lymphoma.
(Dr. Peter Salgo)
So that would be classifying the disease?
(Richard)
Classifying the disease.
(Peter)
Follicular, non-follicular.
(Richard)
orrect.
(Peter)
What else do you have to do before you want to treat somebody?
(Richard)
The first thing you do, is make the diagnosis. The second thing we tell patients, is you do staging. Staging means, where has the disease gone? Where is it localized? What's the extent of the disease?
(Peter)
Okay, and how do you do the staging?
(Richard)
You do it by a variety of things. You use a physical examination. You use x-ray and nuclear medicine tests, so called CT or CAT scans. We now use the thing called PET scans. And we try and find out, is the disease only in her neck? Has it spread to other areas of her body? Sometimes we might also do biopsies elsewhere.
(Peter)
So that, that classification is, what the disease is?
(Richard)
Right.
(Peter)
And staging is, where it is.
(Richard)
Correct.
(Dr. Peter Salgo)
Joel, what was it, how long did it take for you to get your diagnosis, and what did you do during that time?
(Joel Seligman)
It took a few days, and it's interesting, I can't remember precisely how many. And you just sort of put yourself hold. That is, I had confidence in the doctors. I knew they were doing the right diagnostic tests. I knew there was nothing I could do, that was going to make it come faster, and ultimately, was going to hear what it was.
(Peter)
Did you go back to your business, go to work? Were you able to concentrate?
(Joel)
Yeah. And frankly, going to work and...
(Elizabeth)
That's good.
(Joel)
...and distracting myself, if you will, was, was very helpful. You don't want to rush diagnosis. It's wrong. That would be heart breaking. You want to get the real facts, as quickly as is appropriate.
(Peter)
And when you got, after that wait, what you had defined. By the way, what did you have?
(Joel)
I had a B-cell stage I diffuse form of non-Hodgkin's lymphoma. The real point of it is, it's treatable, it was curable. And at that point, you know, there are no guarantees. You know, the doctors who treated me, communicated the diagnosis, prognosis of treatment. And in effect, I became an airplane passenger. And by that, what I meant was, I ultimately developed confidence in the people who were treating me, and my job was to show up and do what they told me.
(Peter)
Sarah's tests showed that she has disease that is limited to "above her diaphragm." What is the diaphragm doing in a discussion of lymphoma here?
(Richard)
It turns out that one of the separations of, I talked about staging, where the disease is. If it's above your diaphragm, which as you know, is kind of at the top part of your abdomen, in general, that's the above, is that, is I stage, and it gets higher stages if it's below that. So it's important to show how far the disease has spread.
(Peter)
And more, the higher the stage, the worse the disease, the worse the prognosis?
(Richard)
Stage I is the best. Stage IV is the most advanced. But, it doesn't mean it's still not curable.
(Peter)
Well, she's told, Sarah does, is that she has IIB non-Hodgkin's lymphoma. Does that make sense to you?
(Richard)
Sure.
(Dr. Peter Salgo)
ll right. Sarah waited a while before coming to see her doctor. Is that going to affect her prognosis statistically? Or since she's not a statistic, is that specific for her? I mean, does it matter if you see someone in two days, two weeks, two months, two years?
(Richard)
Every model shows that treating cancer earlier, as soon as you can make a diagnosis, if you have effective treatment, is the way to go.
(Peter) We've covered an enormous amount of ground here. Let me just sum up a little of what we've been discussing. Lymphoma is a cancer of the lymph glands. Classification tells you what kind you have. Staging tells you where it is. The important thing, I think we've established, is don't ignore the signs or the symptoms. A sign is something that your doctor might notice, a symptom is something that you notice. Early diagnosis can be really critical. We all agree about that.
(All)
Yeah. Absolutely.
(Peter)
All right, let me tell you just a little bit more, about what's going on over here. Sarah now has a diagnosis of non-Hodgkin's lymphoma. Joel, you got all this stuff. All this diagnosis. What was the first thing you did when you heard?
(Joel)
I exhaled because it was stage I. I exhaled because it was treatable. But most of all, you then wrestled with the question that perhaps your show is, probably as focused on as any, which is, do you want a second opinion. And...
(Peter)
Did you?
(Joel Seligman)
I initially didn't. I had great confidence in my doctor. But a couple of people called me and basically said, look. The overwhelming probability is this is going to be fine. You'll be all right. If anything goes wrong, you owe it to yourself and you owe it to your doctor, to know you did everything you could.
(Peter)
What did your doctor say, and were you worried about what his response would be?
(Joel)
I didn't know what his response would be. He was totally professional. It happens all the time. He just suggested I get someone really good, and had a couple of recommendations as to leaders in the field. But I think the patient going through this the first time, is likely to be far more anxious than doctors who are used to a system of second opinions, and understand how important it can be.
(Peter)
Do you guys hear about this concern all the time?
(Dianne Savastano)
I do hear about it all the time, and many people have a lot of anxiety about how their physician will react, and they're very worried about their relationship. They want to have a really positive relationship, and they are fearful that they will offend. So it's really important, I think, if a physician reassures them and says, we do this all the time. It's perfectly normal. I'm glad you're going to have a second opinion.
(Dr. Peter Salgo)
I can tell you, that Sarah did get treatment. Assuming it's a favorable cell type, what kind of treatment would be typical for someone like Sarah?
(Richard)
Well, let me split it in two, because...
(Peter)
Sure.
(Richard)
...I suspect that's not what she had, but it could be. If she had the indolent kind, the slow growing type that we traditionally say is not curable, then you get into a very complicated series of discussions. Some people don't treat them. Some people use antibodies. Some people use chemotherapy. It's a chronic disease, if it's the indolent one. It's one that you can't cure.
(Peter)
Indolent means slow growing.
(Richard)
It means slow growing, and not necessarily curable, but controllable for many, many years. The other side of that coin, are the aggressive lymphomas. The bad new is, they can kill you very quickly, if they are not successfully treated. But the good news is, if they're successfully treated, you can be cured and never have to deal with them again. So, you know, if she had an aggressive lymphoma, we would use chemotherapy, antibodies, and potentially radiation for her, depending on what happened and what the type was.
(Peter)
Well, Sarah got R-CHOP. Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. This is chemotherapy. What's it like?
(Richard)
They kill rapidly dividing cells. That's what most chemotherapy does. So they're a little nonspecific. Because other cells in your body are dividing, and therefore, you can have side effects, because your bone marrow, your gastrointestinal tract, all those cells divide, and that's where the nonspecific side effects occur, as well as the killing of the tumor. The antibody goes directly there, and is a more of a targeted therapy. The data says that the combination of those can cure people with the aggressive form.
(Peter)
Joel, what did you get?
(Joel)
R-CHOP.
(Peter)
You got R-CHOP.
(Joel)
And in exactly the set of drugs you described. The part about the treatment that, I think really deserves comment, is there, now it's almost like an urban myth that you'll have nausea, that you'll have terrible side effects. And at least my experience in that, and the other people in the center where I was being treated, was this was much more manageable, than earlier had been the case. And in a sense, getting the treatment earlier rather than later, should not be deterred because of any fear of the chemotherapy itself.
(Dr. Peter Salgo)
Was it only chemo? Did you get anything else in addition?
(Joel)
Oh, I had radiation.
(Peter)
What's the purpose of the radiation here, if he's already getting R-CHOP?
(Richard)
The chemotherapy and the antibody decrease the tumor burden, decrease the number of tumor cells in the site, and probably the safest way to consolidate and kill the very last cell once you've got it shrunk down, is to radiate that area. And so the radiation is then directed to the lymph nodes that are left in the hope of sterilizing the entire area.
(Peter)
At the end of the day, did they all work?
(Joel Seligman)
So far as I can tell, and they did a subsequent CAT scan and PET scan, and I was cancer free.
(Peter)
Now, I use the word, work, advisedly here. I didn't ask, are you cured? Does this stuff cure it? Or does it just turn it off for a while? What's his, what are the chances here of a complete cure, and what would you, what is a cure anyway? We discussed this a little bit, let's discuss it some more
(Richard)
All right. So let's go back to one of the other discussions, and a little clarification on that five-year survival. The two different lymphomas, the aggressive and the indolent, have different meanings of five-year survival. In the aggressive lymphomas, if you're alive and disease free for five years, you have over a 98% chance of being cured. In the indolent lymphomas, that's not true. So again, if we can get the disease to, the first thing we have to do, is get the disease to go into what we call a complete remission. That means, it goes away by any test we can make. Then we have to follow the patient over time, because cure means ultimately that you have the same survival as someone else exactly like you in the population without cancer. The only way you can tell that, is by following the people for a long time. So you get into a remission, then you get followed. By five years, in the aggressive forms, the odds are really good that you're cured. Not in the indolent, unfortunately, because it could come back after that point.
(Peter)
But you say, unfortunately. Let me ask some population dynamics, and psychodynamics with this. If it is indolent, and it goes on for a long, long time, because it's not growing very fast. Isn't that simply saying you've got a chronic disease? What's the difference for a patient being told, you have chronic hypertension, or a chronic indolent lymphoma that's going to go on for the next 20 years?
(Lou)
Well, cancer has a different tone. I mean...
(Peter)
Is it all tone, though?
(Lou)
It's, well, it's not all tone. With the lymphomas, it depends on the patient. So lymphoma is very age specific, also. So if I have an 85-year-old patient who has fairly severe coronary artery disease and COPD...
(Dr. Peter Salgo)
COPD is lung disease.
(Lou)
Lung disease. They have a number of comorbidities, and they have a fairly indolent, nonaggressive form of lymphoma. The likelihood is that their cancer is going to outlive them. So it, you really have to take the whole package in some respects. And that some of these lymphomas, as I'm sure Rich will say, they can change. Over time they can become aggressive. They do increase your risk for infection, as well, so it's not this free ride, in some respects.
(Dr. Karen Syrjala)
Part of what's occurring in medicine, is that not only is our cure rate improving, but our ability to manage the symptoms, like Joel was talking about, where his symptoms were pretty well managed. It has improved so much, our ability to manage the nausea, the things that people used to be terrified of when they hear the word, cancer, has really changed. And now people can live with cancer, if not comfortably, at least have quality of life.
(Peter) Is it possible to explain to somebody who has just been told here, that she has cancer. That in fact, your life expectancy with an indolent cancer, even though uncurable, may not be all that different, from somebody with chronic heart disease, chronic diabetes, high blood pressure. Is that possible? Can you do that?
(Dianne Savastano)
I think you can do it, but I think you have to do it, and redo it, and redo it, because I think you'll only take in a very small amount of information, and it will need to be repeated the next time, and be repeated the next time. I just think it needs to be a continuous conversation.
(Lou)
It always amazes me to see the patients that have, you know, a cancer that's been recurrent, whether it's lymphoma or some other form, how they learn to live with that.
(Dr. Elizabeth Holley)
There are more than 400,000 people with lymphoma in the U.S. right now, who have the disease in various forms, and they are going about their normal lives and...
(Joel)
Let me say something that is, there can't be too much praise for the doctors and nurses and technologists involved. You know, from the moment you get the diagnosis, you're a cancer survivor. They're on your side. They're doing everything they can for you. When you go in for treatment, you're with people who have similar experience. You compare notes. There's the sense that you're now part of a club you didn't want membership in. But at the same time, it's a club that's mutually quite supportive. And what you find is that people are intrinsically optimistic under such circumstances. They help each other. And you don't sit around talking about mortality data. You sit around talking about getting better.
(Elizabeth)
Right.
(Peter)
The other question I have, is you say there are so many hundred thousand people with this disease. Who are these people?
(Elizabeth)
Well, they're primarily older, about 75% or so of the disease is diagnosed after the age of 55. And they are, if, there's a higher incidence in men than in women. They're people like all of us. Lifestyle factors, some of the risk factors that have been found are, for personal factors that you can actually control, are dietary factors, but not across all studies. Viruses are associated with non-Hodgkin lymphoma. You can't really control those viruses. The United States has the highest rate of non-Hodgkin lymphoma in the world.
(Peter)
What's coming down the pike, in terms of treating this?
(Richard)
A tremendous amount of research going on. The most appropriate one to talk about, first is this antibody, rituximab, which has, for the first time, probably increased the survival in the aggressive form by about 20%, which is not a trivial amount. I mean the cure rate by 20%. And also for the first time, even in the indolent, the patients are now surviving longer with rituximab. But there are many antibodies, we are moving to an area of what we call targeted therapy. That means we have to understand more about the cancer cell and get drugs or treatments that work on the processes of the cancer cell, as opposed to the normal cell. That will minimize the toxicity and give us more specific treatment.
(Peter)
Let's pause for just a minute right now. And to sum up what we've been talking about. The treatments for non-Hodgkin's lymphoma are effective, and they're improving and there's more stuff coming down the pipeline all the time. People can live long and productive lives with the disease. And I'm assuming this is both indolent and aggressive disease, provided you've got the right docs, get the right therapy all the time. Now Joel, you're the president of a major university at a medical center. So you had access to great medical care, but you also on the obverse of that, perhaps, felt compared to go public, right away, with what was going on with your health. What was that like?
(Joel)
I went public for two reasons. First, I felt very strongly that being a president of a university is based upon mutual trust. If I had a life-threatening disease and didn't talk to people and they learned it later, their confidence in me would have been undermined. But there was probably a more important point. And that was, I wanted to encourage people to get an early diagnosis. The truth is, going in to see the doctor earlier, can be tremendously important in saving lives. And I was hopeful that if I could just encourage a few people to do that, it was worth it. It takes no courage to talk about this. There's no shame in having cancer. It's, so far as I can tell, almost randomized. It had nothing to do with my lifestyle, so far as I could tell. But the reality was, I think for faculty, for my students, for those I worked with, I take great pride in the fact that I've always been honest them, and I was able to continue that way.
(Peter)
Well, thank you so much for being here, and all of your great discussion. We are out of time. We covered a lot of ground today. So let me just sum up some of the key things to remember right now. Lymphoma is a cancer of the lymph glands and classification tells you what kind you have, staging tells you where it is. Both are important, they can take some time. The important thing is, don't ignore any signs or symptoms. Early diagnosis can be critical, double negatives notwithstanding. The treatments for non-Hodgkin's lymphoma are effective and improving. People can live long productive lives with this disease. It's important to remember that. And our final message is this. Taking charge of your health means being informed, having quality communication with your doctor. I'm Dr. Peter Salgo, and I'll see you next time for another Second Opinion.
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