Cutaneous T cell lymphoma Q&A: Treatment, misdiagnosis, and advances in research
May 23, 2016 | by City of Hope
Cutaneous T cell lymphoma (CTCL) is a lymphoma that arises in the skin, at first showing up as a skin rash or as lesions on the body that can range from flat lesions to more visible tumors or ulcers. Despite being relatively painless in the early stages of the disease, CTCL can also progress to the blood, inflicting painful rashes that can cover up to 80 percent of the body.
Here, Jasmine M. Zain, M.D., associate clinical professor in the Department of Hematology & Hematopoietic Cell Transplantation at City of Hope, discusses various treatment options, the challenges facing minorities in particular and promising research on the horizon to fight this disease.
What treatment options are currently available for CTCL?
Because it presents itself as a skin disease, a dermatologist is usually the first one who encounters these patients and, at least in the initial stages, it’s often treated like a skin problem: with skin-directed treatments, not chemotherapy.
This can be a very indolent disease. Patients can have it for a very, very long time. In the earliest stages of the disease, there may be just a few patches of rash on the skin. Patients can be treated with things like light therapy, topical chemotherapy or topical steroids.
In addition, there are biologic agents that can be administered intravenously or orally. Finally, there are nonchemotherapy systemic agents that can be used to treat this disease very effectively. Patients can lead a normal life, even though the symptoms of the disease may never be cured.
However, CTCL can also evolve into a more aggressive form. Lesions may progress to the face or scalp and can turn into tumors or ulcers. This can be very disfiguring, and the risk of infection goes up. Ultimately, if the disease becomes more aggressive, a stem cell transplant may be required, but this option is typically reserved for bad cases.
Are minorities more at risk?
Infection with the HTLV-1 virus has been linked with adult T cell leukemia/lymphoma, although most people infected with this virus do not develop lymphoma. This infection is prevalent in parts of Japan and the Caribbean.
This disease also seems to affect African-Americans more frequently, and there have been poorer survival outcomes reported in this population, as well. However, these poorer survival outcomes might also be attributable to limited access to health care.
Because CTCL is a chronic disease, regular access to medical care is needed. Also, because it’s such a rare disease, it’s best treated at a specialized medical care center, but access to these centers can be challenging for some groups of patients.
Treatment for CTCL also requires frequent visits. Even “light” treatment, for example, is done three times per week. A patient needs to make time to do that, and they need transportation. Not to mention, CTCL treatment is not offered everywhere. These kinds of things make it difficult for some groups of patients to access regular therapy.
Is it easy to diagnose?
Not always. Because it’s such a rare disease, misdiagnosis is common, particularly in early stages. CTCL can easily be confused with things like psoriasis or eczema, and it can sometimes take a while for clinician to realize that this could be a T cell lymphoma.
Unless somebody is looking for this, they’re likely not going to find it, particularly in the early stages. Patients get very frustrated, and they go from place to place, until they find somebody who is really is an expert, like at City of Hope.
Is there any promising research underway?
Unfortunately, because this is still not a curable disease, most patients will eventually run out of treatment options. Thus, we have a lot of ongoing research at City of Hope to fight, and eventually cure, CTCL, and we are trying to open new trials.
One area of research that’s being pursued is in immunomodulatory agents that modify the immune system to fight lymphoma. Another area is trying to modify the microenvironment around the tumor to help treat the disease. Christiane Querfeld, M.D., Ph.D., a dermatologist and dermatopathologist at City of Hope, is working in this field, and we have several trials that will soon emerge from her research.
For advanced patients, we can do stem cell transplants to try to provide curative therapy, and there is research underway to improve outcomes here, as well.
What else should people should know about this disease?
If I were to send one message, it would be the following: If you’ve been diagnosed with this disease, try to find a center that has expertise in treating CTCL, at least to get a second opinion. I’ve seen several cases where people have received chemotherapy for what was essentially a pimple. There is a lot of mistreatment out there, so getting a second opinion is a must.
Ideally, patients should be seen by both a dermatologist and an oncologist - at City of Hope, we certainly offer that model - but, at the very least, patients should try to find a doctor who’s familiar with this disease.
Finally, if your insurance company is giving you a hard time about it, try to advocate for the fact that this is a rare disease that not everyone knows how to treat. Because it’s a rare disease, people should fight hard to find a place where there’s expertise in order to get the optimal care they need.
Learn more about City of Hope's lymphoma program. If you are looking for a second opinion or consultation about your treatment, request an appointment online or contact us at 800-826-HOPE. Please visit Making Your First Appointment for more information.
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