Cutaneous T cell lymphomas are types of non-Hodgkin lymphoma that arise when infection-fighting white blood cells in the lymphatic system – called lymphocytes – become malignant and affect the skin.
Photo therapy to topical creams, the first defense is to relieve the itching.
Is it eczema? Is it psoriasis? When do you suspect CTCL.
Mycosis fungoides is the most common type of CTCL.
A primary symptom is a rash that arises initially in areas of the skin that are not normally exposed to sunlight.
Listen in as Christiane Querfeld, MD and Melanie Cole, MS discuss CTCL, it's diagnoses and treatments.
Selected Podcast
Cutaneous T Cell Lymphoma (CTCL)
Featured Speaker:
Christiane Querfeld, MD, PhD
Christiane Querfeld, M.D., Ph.D., is a board-certified dermatologist and dermatopathologist with advanced fellowship training in cutaneous lymphoma. Her clinical practice focuses on the diagnosis and management of patients with cutaneous lymphoma and the care and management of patients affected by cutaneous complications of hematopoietic stem cell transplantation. Transcription:
Cutaneous T Cell Lymphoma (CTCL)
Melanie Cole (Host): If you’ve been diagnosed with cutaneous T-cell lymphoma, you might wonder what that means in terms of treatment and outcome. My guest today is Dr. Christiane Querfeld. She is the director of the cutaneous lymphoma program at City of Hope. Welcome to the show, Dr. Querfeld. Tell us a little bit about cutaneous T-cell lymphoma. What is it and how is it diagnosed? What makes it so difficult to diagnose?
Dr. Christiane Querfeld (Guest): Cutaneous T-cell lymphoma is mainly so called mycosis fungoides, and it’s really extremely difficult to diagnose because this T-cell lymphoma can mimic many other benign skin rashes such as psoriasis or eczema. Even if patients have a longstanding rash, they may not be diagnosed because the biopsy does not show features of a T-cell lymphoma and can also on the biopsy look like psoriasis or look like eczema. So you have to have a very, very low threshold as a clinician and as a pathologist to diagnose this type of lymphoma. In particular, you have to make sure that you take a good history. So if it’s a longstanding rash for many, many years, if it’s typically distributed or started in the bathing suit areas, like the buttocks, the lower back, the breasts, the inner arms, all these sun-protected areas, that’s very suspicious for mycosis fungoides. Patients have been in the past before I even see them, they have been treated with topical steroids, and it’s not helping. The rashes always come back. This is also another sign that something is not right.
Melanie: Dr. Querfeld, once it’s diagnosed, what are the first line of defense treatments for CTCL?
Dr. Querfeld: First line treatment, depending on how widespread the lymphoma is. If it’s just the skin, usually you start with so-called skin-directed treatment. And skin-directed treatments is the spectrum of phototherapy, can be PUVA, it can be narrow-band UVB or just UVB. It can be topical steroids in combination or alone before the therapy, topical nitrogen mustard, which is a topical chemotherapy, topical bexarotene, or other topical retinoids, which also affects the malignant cells in the skin, and radiation therapy. This radiation therapy is called electron beam radiation, which is very, very superficial. Electron beam radiation is not the same like phototherapy. Phototherapy is something that’s in the spectrum of the natural light treatment. It’s just filtered. It could be either UVA, UVB, or a certain spectrum from UVB, which is called narrow-band UVB. UVA is not given just as treatment. It’s given with a medication, a pill that sensitizes the body to the light, and the medication is called Psoralen and if you give this in combination with UVA, then it’s called PUVA.
Melanie: When we talk about the skin-directed therapies, Dr. Querfeld, tell us a little bit about the side effects. If we’re going to start with a topical corticosteroid or the retinoids that you discussed, what are some of the side effects, and what do people do about things like itching and the dryness that comes with these skin therapies?
Dr. Querfeld: Side effects. Of course, every treatment has its pros and cons. If you start with topical steroids, usually you give more potent steroids. If you give it for a long, long time and throughout the body, you have to make sure that you do not absorb a significant amount of steroids that can suppress your own body’s production of steroids. Steroids are very helpful and necessary, but you don’t want to suppress your own body’s production. Also, skinning can happen. It’s usually that’s why we put patients on and off to make sure that this does not happen. Certain areas cannot be treated with very strong steroids, such as the private parts or the axillary areas or skin folds, because the skin is thinner and the absorption is higher. Also on the face, you can’t really use high potent steroids. I usually start steroids as first line and give additional treatment once we have established the diagnosis. The phototherapy—in particular, PUVA—can cause skin cancer, other forms of skin cancer like basal cell, squamous cell cancer, but only if you give it for a long, long time. The best pattern or type of phototherapy would be narrow-band UVB. The risk is extremely low. Topical nitrogen mustard and topical retinoids, they can cause skin irritation that you get, like a contact allergy or, really, rashes from the medication. That’s why once we give treatment, I’d like to see my patients very closely and follow up every three to four weeks and see how the treatment works and how we can handle the side effects. One important thing is…
Melanie: What do you typically do about those side effects for people? Can they use over-the-counter creams for the itching, for these agents? What do they typically do to manage and live with this so they’re not so uncomfortable?
Dr. Querfeld: For the itching, it’s adamant to have a good care. And I have a list of eight to 10 unscented, fragrance-free moisturizing creams and ointments. I call it Dr. Q’s top 10. I want to make sure the patients do not use any lotions which are alcohol-based and can contribute to irritation. I do not like any scents in the moisturizers that can cause irritation. If the rashes are very bad, sometimes bacteria can hide in those rashes on the skin. I have implemented -- I like to use so-called bleach baths, which I have adopted from children who are treated for eczema. A quarter of a cup in a full bath once or twice a week, that works wonder. For the itching, moisturizers is the first, mild soap then moisturizing. Second, we could give systemic therapies for itching, like the spectrum of anti-itch medications. I don’t like to go into detail right now because every patient may respond differently. We have also an upcoming trial on patients who are really itchy to see if this new topical formulation can stop the itching. I do have to mention the itching is the major problem in patients who have really the itch. They’re not really bothered by the rash, it’s more the itching. If it’s really severe, such as in Sezary syndrome, it’s really disabling. So we work on it. I cannot promise really wonders, but on a continuous basis, we can decrease the itch significantly. The overall goal is to clear it, and once the patient is cleared, then the itch stops
Melanie: Now, tell us a little bit about phototherapy, the light therapy. What is it intended to do? Generally, what is the outcome for people with CTCL?
Dr. Querfeld: Phototherapy is one of the mainstay treatments, as I mentioned before, and there are experiences, probably 30 to 40 years. It was in particular discovered in the late ‘70s that patients respond very well. How does this work? In two ways. One is that the light can directly kill those atypical, those malignant cells in the skin, and they can also diminish other immune cells who live in the skin, such as dendritic cells, Langhans cells. These are the ones who actually support the growth of the lymphoma cells, and they are all affected by the light therapy. They don’t like the light therapy, and so they eventually die off. Studies have actually shown that if you treat until you clear and then you taper slowly off over months, the recurrence of the lymphoma is less compared when you just stop once the skin is clear. I like to do this, too, like tapering patients off slowly after the skin is completely clear. Usually, what you expect is that you give it about probably around three or four months. That’s the average time patients clear. And then you slowly taper from three times a week to two times a week to once a week and to once every 10 days. This is the process that goes over nine to 12 months, but that may be different. And some patients may just like to be on a maintenance treatment for a year or two every 10 days and would be fine.
Melanie: Dr. Querfeld, in just the last few minutes, please give your best advice for those living with CTCL in managing their symptoms and why they should come to City of Hope for their lymphoma care.
Dr. Querfeld: We have a dedicated multidisciplinary clinic for CTCL or for cutaneous lymphoma. It’s Dr. Zain and myself and Dr. Rosen. We have extensive experience in treating those patients. Myself, I have been involved with care for 14 years, and I’m very fortunate to do this. We have the necessary equipment here. We have the staff who can support us in treating those patients.
Melanie: Thank you so much. You’re listening to City of Hope Radio. For more information, you can go to cityofhope.org. That’s cityofhope.org. This is Melanie Cole. Thanks so much for listening and have a great day.
Cutaneous T Cell Lymphoma (CTCL)
Melanie Cole (Host): If you’ve been diagnosed with cutaneous T-cell lymphoma, you might wonder what that means in terms of treatment and outcome. My guest today is Dr. Christiane Querfeld. She is the director of the cutaneous lymphoma program at City of Hope. Welcome to the show, Dr. Querfeld. Tell us a little bit about cutaneous T-cell lymphoma. What is it and how is it diagnosed? What makes it so difficult to diagnose?
Dr. Christiane Querfeld (Guest): Cutaneous T-cell lymphoma is mainly so called mycosis fungoides, and it’s really extremely difficult to diagnose because this T-cell lymphoma can mimic many other benign skin rashes such as psoriasis or eczema. Even if patients have a longstanding rash, they may not be diagnosed because the biopsy does not show features of a T-cell lymphoma and can also on the biopsy look like psoriasis or look like eczema. So you have to have a very, very low threshold as a clinician and as a pathologist to diagnose this type of lymphoma. In particular, you have to make sure that you take a good history. So if it’s a longstanding rash for many, many years, if it’s typically distributed or started in the bathing suit areas, like the buttocks, the lower back, the breasts, the inner arms, all these sun-protected areas, that’s very suspicious for mycosis fungoides. Patients have been in the past before I even see them, they have been treated with topical steroids, and it’s not helping. The rashes always come back. This is also another sign that something is not right.
Melanie: Dr. Querfeld, once it’s diagnosed, what are the first line of defense treatments for CTCL?
Dr. Querfeld: First line treatment, depending on how widespread the lymphoma is. If it’s just the skin, usually you start with so-called skin-directed treatment. And skin-directed treatments is the spectrum of phototherapy, can be PUVA, it can be narrow-band UVB or just UVB. It can be topical steroids in combination or alone before the therapy, topical nitrogen mustard, which is a topical chemotherapy, topical bexarotene, or other topical retinoids, which also affects the malignant cells in the skin, and radiation therapy. This radiation therapy is called electron beam radiation, which is very, very superficial. Electron beam radiation is not the same like phototherapy. Phototherapy is something that’s in the spectrum of the natural light treatment. It’s just filtered. It could be either UVA, UVB, or a certain spectrum from UVB, which is called narrow-band UVB. UVA is not given just as treatment. It’s given with a medication, a pill that sensitizes the body to the light, and the medication is called Psoralen and if you give this in combination with UVA, then it’s called PUVA.
Melanie: When we talk about the skin-directed therapies, Dr. Querfeld, tell us a little bit about the side effects. If we’re going to start with a topical corticosteroid or the retinoids that you discussed, what are some of the side effects, and what do people do about things like itching and the dryness that comes with these skin therapies?
Dr. Querfeld: Side effects. Of course, every treatment has its pros and cons. If you start with topical steroids, usually you give more potent steroids. If you give it for a long, long time and throughout the body, you have to make sure that you do not absorb a significant amount of steroids that can suppress your own body’s production of steroids. Steroids are very helpful and necessary, but you don’t want to suppress your own body’s production. Also, skinning can happen. It’s usually that’s why we put patients on and off to make sure that this does not happen. Certain areas cannot be treated with very strong steroids, such as the private parts or the axillary areas or skin folds, because the skin is thinner and the absorption is higher. Also on the face, you can’t really use high potent steroids. I usually start steroids as first line and give additional treatment once we have established the diagnosis. The phototherapy—in particular, PUVA—can cause skin cancer, other forms of skin cancer like basal cell, squamous cell cancer, but only if you give it for a long, long time. The best pattern or type of phototherapy would be narrow-band UVB. The risk is extremely low. Topical nitrogen mustard and topical retinoids, they can cause skin irritation that you get, like a contact allergy or, really, rashes from the medication. That’s why once we give treatment, I’d like to see my patients very closely and follow up every three to four weeks and see how the treatment works and how we can handle the side effects. One important thing is…
Melanie: What do you typically do about those side effects for people? Can they use over-the-counter creams for the itching, for these agents? What do they typically do to manage and live with this so they’re not so uncomfortable?
Dr. Querfeld: For the itching, it’s adamant to have a good care. And I have a list of eight to 10 unscented, fragrance-free moisturizing creams and ointments. I call it Dr. Q’s top 10. I want to make sure the patients do not use any lotions which are alcohol-based and can contribute to irritation. I do not like any scents in the moisturizers that can cause irritation. If the rashes are very bad, sometimes bacteria can hide in those rashes on the skin. I have implemented -- I like to use so-called bleach baths, which I have adopted from children who are treated for eczema. A quarter of a cup in a full bath once or twice a week, that works wonder. For the itching, moisturizers is the first, mild soap then moisturizing. Second, we could give systemic therapies for itching, like the spectrum of anti-itch medications. I don’t like to go into detail right now because every patient may respond differently. We have also an upcoming trial on patients who are really itchy to see if this new topical formulation can stop the itching. I do have to mention the itching is the major problem in patients who have really the itch. They’re not really bothered by the rash, it’s more the itching. If it’s really severe, such as in Sezary syndrome, it’s really disabling. So we work on it. I cannot promise really wonders, but on a continuous basis, we can decrease the itch significantly. The overall goal is to clear it, and once the patient is cleared, then the itch stops
Melanie: Now, tell us a little bit about phototherapy, the light therapy. What is it intended to do? Generally, what is the outcome for people with CTCL?
Dr. Querfeld: Phototherapy is one of the mainstay treatments, as I mentioned before, and there are experiences, probably 30 to 40 years. It was in particular discovered in the late ‘70s that patients respond very well. How does this work? In two ways. One is that the light can directly kill those atypical, those malignant cells in the skin, and they can also diminish other immune cells who live in the skin, such as dendritic cells, Langhans cells. These are the ones who actually support the growth of the lymphoma cells, and they are all affected by the light therapy. They don’t like the light therapy, and so they eventually die off. Studies have actually shown that if you treat until you clear and then you taper slowly off over months, the recurrence of the lymphoma is less compared when you just stop once the skin is clear. I like to do this, too, like tapering patients off slowly after the skin is completely clear. Usually, what you expect is that you give it about probably around three or four months. That’s the average time patients clear. And then you slowly taper from three times a week to two times a week to once a week and to once every 10 days. This is the process that goes over nine to 12 months, but that may be different. And some patients may just like to be on a maintenance treatment for a year or two every 10 days and would be fine.
Melanie: Dr. Querfeld, in just the last few minutes, please give your best advice for those living with CTCL in managing their symptoms and why they should come to City of Hope for their lymphoma care.
Dr. Querfeld: We have a dedicated multidisciplinary clinic for CTCL or for cutaneous lymphoma. It’s Dr. Zain and myself and Dr. Rosen. We have extensive experience in treating those patients. Myself, I have been involved with care for 14 years, and I’m very fortunate to do this. We have the necessary equipment here. We have the staff who can support us in treating those patients.
Melanie: Thank you so much. You’re listening to City of Hope Radio. For more information, you can go to cityofhope.org. That’s cityofhope.org. This is Melanie Cole. Thanks so much for listening and have a great day.