Mycosis Fungoides Sezary Syndrome is relatively uncommon and a fast-growing cancer. This cancer affects both skin and blood. Sezary syndrome might appear like eczema or other common skin conditions in the early stages. Doctors say it might be associated with a virus, genetics, or exposure to a chemical. More often, people in their 50s and 60s are more susceptible to this condition. Men are twice at risk of the syndrome than women.
A typical healthcare practitioner may not treat cancer. However, they can offer treatments and medications to improve your mycosis fungoides sezary syndrome symptoms. This guide walks you through everything related to mycosis fungoides sezary syndrome, symptoms, diagnosis, treatment, and more.
What are the Signs of Mycosis Fungoides Sezary Syndrome?
The most visible sign of this syndrome is a distinctive red rash. It hurts, itches, and spreads too swiftly. The earliest symptoms may appear similar to psoriasis or eczema. When the syndrome worsens, the sufferer may develop small, raised bumps on the skin. There can also be chances your skin looks harder or thicker than others (plaque). The person may potentially get skin tumors. Other symptoms might be:
- Fatigue
- Eyelids that turn outward
- Swelling in your lower legs
- Unexplained weight loss
- Swollen lymph nodes
- Hair loss
- Fever
How Is A Sezary Syndrome Diagnosed?
A doctor will carry out a physical test and evaluate your signs. In addition to that, they will ask questions about your health history. If they say that you are suffering from Sézary syndrome, here are the tests that they might conduct:
Blood Tests
In a blood test, along with a complete blood count with a differential, the percentages of various white blood cells will be checked. It even includes a sezary blood count to check the number of sezary cells in the blood and a peripheral blood smear.
Biopsies
The healthcare provider might also carry out the biopsies of your skin, bone marrow, or lymph nodes.
Immunotyping
Immunotyping examines cell surfaces for indicators to detect various forms of lymphomas. For performing immunotyping, the provider uses a blood or tissue sample.
Other Tests
If blood and lab testing reveal the signs of Sézary syndrome, the doctor may carry out other tests to check whether the cancer has spread. Chest X-rays, CT scans, and positron emission tomography (PET) scans are some of the probable tests.
Mycosis Fungoides Vs. Sezary Syndrome
Mycosis fungoides and Sézary syndrome are two different cutaneous T-cell lymphoma types. In terms of symptoms, both conditions are almost the same. But, mycosis fungoides show more red skin rashes compared to sézary syndrome.
Sézary syndrome is also believed to be a variant of mycosis fungoides, even though they are two different forms of conditions. T-cell lymphocytes turn cancerous and impact skin cells in both cases. The signs of mycosis fungoides only show on the skin.
If the patient’s blood has cancerous T-cells, they are known as Sézary cells. The condition is referred to as Sézary syndrome. Sézary syndrome involves the skin and cells that move through the bloodstream. Cancer cells can be detected in lymph nodes and other tissues of the tissues.
Areas Where Mycosis Fungoides Sezary Syndrome Occurs
People with mycosis fungoides sezary syndrome often develop skin rashes from head to toe. Treatment may become necessary in this situation. The most common areas of mycosis fungoides sezary syndrome occurrence can be:
- Upper thighs
- Armpits
- Crook of the elbow
- Breasts
- Groin
It may be quite rare, but the cancerous cells can spread to organs like the spleen, lungs, liver, and lymph nodes.
What Are The Causes Of Mycosis Fungoides Sezary Syndrome?
As per research, Sézary syndrome affects 0.1 per million people. Cutaneous T-cell lymphomas impact about ten people in a million.
What exactly causes Sézary syndrome is still unexplored. But, the majority of people who develop the syndrome experience chromosomal defects in the DNA of cancer cells. These anomalies are not there in healthy cells.
A majority of people suffering from mycosis fungoides sezary syndrome experience at least one chromosomal abnormality. This may result in genetic hiccups that allow cell growth and multiplication without control. Usually, somebody who has mycosis fungoides has additional DNA in regions of chromosomes 7 and 17. Other times, they might suffer a loss of DNA from regions of chromosomes 9 and 10.
Treatment For Mycosis Fungoides Sezary Syndrome
Here are some effective treatments your doctor may recommend for Mycosis Fungoides Sezary Syndrome. But that may depend on your signs and symptoms. The treatments can be:
Radiation Therapy
The therapy includes external radiation therapy or complete skin electron beam radiation therapy. Radiation therapy utilizes high-energy X-rays and other sorts of radiation to combat cancerous cells. Healthcare professionals sometimes use total skin electron beam radiation therapy for treating Mycosis Fungoides Sezary Syndrome.
Total skin electron beam radiation therapy is a sort of external radiation therapy machine that directs electrons to the whole body’s skin. One can go for external radiation treatment to receive palliative care and reduce symptoms. Therefore, it can ultimately improve the quality of life.
Chemotherapy
Healthcare practitioners may employ topical chemotherapy, which is administered to the skin, or systemic chemotherapy. This affects the entire body of the patient. A systemic chemotherapeutic treatment is a vorinostat.
Chemotherapy uses drugs to prevent the occurrence of cancer-developing cells. They either combat these cells or stop them from spreading or multiplying. If the chemotherapy is given externally or orally, the drugs will either go through the bloodstream or may reach the cancerous cells.
Phototherapy
Providers may even use extracorporeal photopheresis to treat the condition. This entails the collection of white blood cells (WBCs) and exposure of the cells to UV light. Photopheresis or UV phototherapy can also be used.
In phototherapy, light waves are used to cure the condition. The skin of the patient will face ultraviolet rays for a specified amount of time. The UV rays will shut down the skin’s immune system cells. Before the therapy, the doctor would ask you to remove any clothing covering your affected skin area. The areas which don’t need phototherapy must be fully covered.
Targeted Therapy
Providers may use targeted therapy to cure the condition. Monoclonal antibody therapy is an example of targeted therapy. They will use certain drugs or other substances to detect and eradicate cancerous cells. In general, compared to chemotherapy or radiation therapy, targeted therapies may result in damaging normal cells.
Immunotherapy
The doctors use the patient’s immune system to combat cancer during immunotherapy. Our body offers protection against cancer with its natural defenses. They strengthen, direct, or repair using substances produced by the body or in a laboratory. Immunotherapy is a type of biological therapy. A few biological therapies stimulate or suppress the patient’s immune system to aid the body in combating cancer. Others attack certain cancerous cells to stop their growth. These therapies can even alleviate the adverse effects that some cancer treatments incur.
Other Drug Therapies
The other potential treatments include the use of drugs like corticosteroids, retinoid creams, or gels like bexarotene. The practitioners use topical corticosteroids to ease signs like skin swelling or inflammation. These are available in the form of lotion, ointment, or cream. Lenalidomide is another medication that aids the immune system in warding off aberrant blood cells or cancerous cells. This may even stop tumors from growing new blood vessels.
Also Read: Graft Vs Host Disease: Causes, Diagnosis, Treatment, and More
What Are Various Stages Of Mycosis Fungoides Sezary Syndrome?
These are the several stages of Mycosis Fungoides Sezary Syndrome that you should be aware of:
Stage 1
In the first stage of mycosis, fungoides emerge on the skin, covering nearly 10 percent of the skin’s surface.
- Stage 1A: Fewer than 10 % of the body is covered by patches or plaques
- Stage 1B: About 10 % or more of the body is covered by patches or plaques.
Stage 2
You can see plaques in the skin along with patches and the likelihood of tumor development.
- Stage 2A: No tumors are present on the skin
- Stage 2B: One or more tumors present on the skin
Stage 3
Tumors are visible on the skin’s surface. These are accompanied by lymph node amplification and general symptoms. This shows that the cancer has spread to other organs.
Over 80 % of the skin surface turns red in the third stage of Mycosis Fungoides Sezary Syndrome. The patient might see patches, plaques, bumps, or tumors. Moreover, there can be a reduction in the number of Sézary cells in the blood.
- Stage 3 A: There are no cancerous cells in the blood.
- Stage 3 B: There are fewer cancer cells in the blood.
Stage 4
In this stage, the majority of the skin is affected. The person has a high number of Sezary cells, or the cancer has spread.
- Stage 4A1: The lymph nodes expand, but they aren’t cancerous. A large number of sezary cells are also there in the blood.
- Stage 4A2: There can be cancer in the lymph nodes. However, they may not spread to other body parts.
- Stage 4B: The cancer has grown to other organs, such as the spleen or the liver.
Who Is More At Risk Of Mycosis Fungoides?
Often, Mycosis Fungoides occurs in individuals above 60 years of age. But it can happen to anyone, regardless of age. Compared to women, this condition is more common in men. If we talk about skin color, Mycosis Fungoides is very common in black people.
How Common is this Condition?
Mycosis fungoides is an uncommon disease. The number of new cases identified each year is quite low, around 3000. Due to patients’ extensive longevity, there may be as many as 30,000 patients in the US and Canada.
As we have mentioned earlier, mycosis fungoides is more prevalent in men than in women, in blacks than in whites, and in individuals over the age of 50 compared to youngsters. These figures are likely low estimates due to:
- The challenges of detecting the disease in its early stages
- The slow progression of mycosis fungoides
- The lack of an accurate reporting system
Questions To Ask The Doctor Before The Treatment
You must prepare a list of questions to ask your doctor before the treatment for Mycosis Fungoides. The list should have questions like:
- What will be the potential side effects of the treatment?
- Will the condition return even after the treatment?
- How do we lessen the chance of mycosis fungoides recurring?
- What can I do to care for myself during the mycosis fungoides treatment?
Survival Rate For Mycosis Fungoides Sezary Syndrome
The early stage of Mycosis Fungoides Sezary Syndrome reveals the longest survival rate. As per a meta-analysis of 7279 patients suffering from Mycosis Fungoides Sezary Syndrome, it was found that 10 to 20 % of stage 1B patients survive less than five years. On the contrary, in advanced-stage Mycosis Fungoides patients, there is a 5-year survival chance, which is around 20 to 60%.
Is Mycosis Fungoides Sezary Syndrome Curable?
The bad news is that no cure for this syndrome exists. However, some therapies can aid in managing the signs and symptoms. The therapies can also keep abnormal cells from spreading and decelerate the growth of cancer.
Follow-Up Treatment For Mycosis Fungoides Sézary Syndrome
The patients should be proactive in their healthcare both during and after treatments. They should not neglect to maintain a master file of healthcare records, report new symptoms, and ask questions.
Those who have a habit of smoking must quit for their betterment after the treatment. The follow-up visits for sézary syndrome patients will rely on the stage of the disease and treatment. Furthermore, it may range from a couple of weeks while starting new drugs that need monitoring once every six months.
Blood tests, physical examinations, and sometimes imaging tests like PET or CT scans are generally there in follow-up visits. Along with determining ailment recurring, follow-up care may help detect and treat rare treatment side effects.
What If Mycosis Fungoides Recurs?
If mycosis fungoides come back post-treatment, it is a relapse. The cancer can recur in a similar area to the original tumor or a new site. It is common to have relapses.
When the condition returns, your healthcare provider might review their previous approach on the basis of the person’s wants and goals. A person might wish to carry on with treatment to extend their lifespan. Others may emphasize symptom alleviation as well as supportive care.
Conclusion
Mycosis Fungoides Sezary Syndrome might be an aggressive cancer. So, you must visit an expert physician and get therapy. This will ensure you get the ideal control of your signs and the best possible prognosis.
Are you having mycosis fungoides sezary syndrome symptoms? Do you need proper guidance on how to deal with the condition? At University Cancer Centers, you can expect to get the same. We are a safe haven for every cancer patient out there. From precise diagnosis to necessary assistance, we are there for you.
