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Only skin eruptions are visible on the outside, which is why some people think it is a skin disease. It looks unsightly, so patients are stigmatized … However, under skin eruptions and sometimes oozing wounds, there is a dangerous, little known and rare disease – cutaneous T-cell lymphoma (CTCL), also known as cutaneous T-cell lymphoma.
We associate lymphomas, or cancers of the lymphatic system, with enlarged nodes, not skin lesions. However, this lymphoma develops primarily in the skin and not in the lymph nodes. This is one of the 40 types of non-Hodgkin’s lymphoma. It is a cancer in the course of which there is an uncontrolled growth of T lymphocytes, i.e. cells that guard our immunity and are to eliminate possible enemies that threaten our body.
Can deal with internal organs
In cutaneous T-cell lymphoma, the primary site of the neoplastic process is the skin, namely the lymphocytes in the skin. Unfortunately, the disease is not limited to our “outer shell” – through the lymphatic system it can spread to lymph nodes through the lymphatic infiltrate, and also affect internal organs.
CTCL lymphoma is not a homogeneous disease. The most common types of this cancer are mycosis fungoides (MF), Lymphomatoid papulosis (a very mild course of the disease) and primary cutaneous anaplastic large T cell lymphoma (pcALCL).
Here, as in most neoplastic diseases, time is of the essence. First of all, the time when the cancer will be diagnosed. This is not good because the disease is very rare and is often confused with hives or psoriasis. Sometimes patients have to visit several specialists over the course of many years to be correctly diagnosed. Meanwhile, the quick implementation of the correct oncological treatment gives patients a chance for a long-term lack of disease development and improves their quality of life.
Sometimes the cause is hard to find
What is the cause of this cancer? It is not known exactly. Experts talk about the possible influence of environmental, genetic and immunological factors. It may be, for example, many years of occupational exposure to contact with chemicals, but also an established autoimmune disease, urticaria or atopic dermatitis. Among potential causes, specialists also mention taking immunosuppressive drugs, previous oncological treatment, but also some bacterial or viral infections. However, in many patients it is difficult to find the cause of the disease because the above-mentioned factors are absent.
What are the symptoms of this lymphoma? Completely different than in other lymphomas. Already at the beginning of the disease, itching and skin eruptions appear. However, they are not specific enough to recognize the disease at first glance. It is at the beginning of the disease that diagnostic errors and suspicions of atopic dermatitis, psoriasis or allergic reactions occur.
Struggling with pain
When the disease is more advanced, the lesions affect the lymph nodes and are located in organs other than the skin. Then the patients develop ulcers, secondary bacterial infections on skin lesions, enlarged lymph nodes. As much as 40 percent. patients suffer from pain, often resulting in sleep disturbances. These patients also experience problems with movement and have difficulties with the proper functioning of the hands. As a result, they often have to quit their jobs.
People struggling with cutaneous T-cell lymphoma often withdraw from social life because they cannot accept their appearance. And it is no surprise, considering that everyone now requires us to be beautiful, young and healthy. Erythematous-exfoliating lesions, infiltrative, blue-red tumors with a tendency to form massive ulcers, this is not something that helps in self-acceptance and acceptance by the society, and it must be remembered that it is an everyday reality of these patients.
This atypical lymphoma is not a cancer disease that quickly destroys the body and leads to death. That is good, of course. However, its slow, long-term course makes it very important to provide these patients with a good life, free from pain and suffering.
Diagnosed by a dermatologist
If the disease is manifested by skin lesions, but it is not really a skin disease, who and how can diagnose it? However, a dermatologist, in collaboration with a pathologist. The examination that allows the diagnosis of the disease is a histopathological assessment of the skin, sometimes supplemented by a histopathological assessment of the affected lymph node or the part of the organ in which the disease has developed. The test also tells you which CTCL lymphoma you are dealing with exactly – T, B or NK cells.
If the histopathological assessment does not give an unambiguous answer, auxiliary tests are performed, e.g. molecular tests, imaging tests: X-ray, computed tomography. During the diagnosis, the clinical advancement of skin lesions and their extent are also assessed. It also influences the selection of the appropriate therapy and the determination of the prognosis.
Treatment depending on the advancement of the lesions
Depending on the subtype of this lymphoma and the stage of the disease, the doctor selects the appropriate therapy. It can be local treatment, phototherapy, radiotherapy and systemic treatment in the case of an advanced disease process.
Younger patients, in good general condition, with advanced disease, may benefit from one more therapeutic method – allogeneic hematopoietic stem cell transplantation. This treatment method can be used when others fail.
PL/ADC/20/0055