Sézary syndrome is a T-cell lymphoma and manifests itself in swelling of the skin, itching and scaling, among other things. The exact circumstances of its development are not yet clear, which makes treatment and prevention more difficult.
What is Sézary Syndrome?
Sézary ( Baccaredda ) syndrome belongs to the group of T-cell lymphomas. Lymphoma is an abnormal enlargement of the lymph nodes, which can mean both swelling and a tumour. The syndrome got its name from the French dermatologist of the same name. Sézary syndrome is a cutaneous T-cell lymphoma that affects certain cells of the immune system. Most of those affected are over 50 years old. Men are more likely to develop Sézary syndrome than women. For what is craniomandibular dysfunction, please visit gradinmath.com.
The cause of Sézary syndrome lies in the T cells. T cells are special cells of the immune system that belong to the white blood cells. In Sézary syndrome, the T cells are altered and falsely trigger a defense reaction of the immune system, which mainly results in various symptoms of inflammation. The nucleus of the mono- and lymphocytoid cells is large and appears folded. In addition, in Sézary syndrome, these cells are characterized by a narrow rim of cytoplasm.
The cell may have several vacuoles that are grouped around the cell nucleus and appear like a necklace. The signs are thus very similar to the picture that is also seen in another form of T-cell lymphoma, namely mycosis fungoides. Special tests can detect these abnormal cells in Sézary syndrome in the skin, bone marrow and blood. For diagnosis, physicians usually consult a blood and/or skin test.
Symptoms, Ailments & Signs
The most noticeable symptom of Sézary syndrome is the widespread reddening of the skin, which doctors call erythroderma. Erythroderma is an inflammatory reaction in the human body that manifests itself superficially in the form of reddened skin all over the body. It usually causes affected tissues to lose salt, protein, and fluid, which increases the risk of infection.
In the case of Sézary syndrome, medicine also speaks of malignant reticulaemic erythroderma. It can take the form of pyoderma, which is a purulent, burning inflammation that is another symptom of Sézary syndrome. Other signs of the disease are itching, flaking or keratinization of the skin, which medicine calls hyperkeratosis. Hyperkeratosis makes the outermost layer of skin, the striatum corneum, thicker as more keratinocytes or corneocytes form.
Hair loss all over the body can also be a potential sign of Sézary syndrome. The disease can also cause semi-annular wrinkling. The skin hangs down in typical places such as the elbows or buttocks because the tissue no longer holds it taut. The result is a pattern that is vaguely reminiscent of cellulite but spreads more evenly across the skin. There is also typically swelling of the lymph nodes, and occasionally changes to the fingernails and toenails can appear.
Diagnosis & course of disease
In addition to the skin symptoms, increased lymphocyte values can provide an initial clinical indication. Examination of the blood and the cells it contains can bring clarity. In the laboratory test, the PAS reaction is crucial. The “Periodic acid-Schiff reaction” is a staining technique that makes carbohydrates visible under the microscope. The stained parts of the cell then appear in the blue-violet color spectrum and can thus be more clearly distinguished from one another.
In the differential diagnosis, physicians primarily have to rule out neurodermatitis, psoriasis and mycosis fungoides. The latter is a disease that also belongs to the T-cell lymphomas and therefore has a very similar clinical picture. Mycosis fungoides occurs more frequently than Sézary syndrome and forms tissue nodules.
Patients with Sézary syndrome suffer from various skin disorders. In most cases, this also results in significantly reduced aesthetics, so that most patients are ashamed of the symptoms and do not feel comfortable with them. This can lead to depression and inferiority complexes.
Patients suffer from itching and reddening of the skin. The skin itself can flake severely or be affected by a rash. Scars often form as a result of constant scratching. In many cases, Sézary syndrome also leads to hair loss that occurs all over the body. Young people in particular can become victims of bullying or teasing and also suffer from mental disorders.
Sézary syndrome can also discolor or change the nails in general. In most cases, the symptoms can be treated well. Taking vitamin A also has a very positive effect on the disease and can limit the symptoms. There are no particular complications. Furthermore, the life expectancy of the patient remains unchanged by the Sézary syndrome.
When should you go to the doctor?
In any case, the person affected with Sézary syndrome is dependent on medical treatment by a doctor. It cannot heal itself, so treatment by a doctor must be carried out in any case. The sooner the doctor is consulted, the better the further course of this disease. A doctor should therefore be consulted as soon as the first signs and symptoms of Sézary syndrome appear. The doctor should be consulted if the person concerned suffers from reddening of the skin that occurs for no particular reason and does not go away on its own.
It spreads all over the body and leads to a significant reduction in aesthetics. Furthermore, severe itching or the formation of scales can indicate Sézary syndrome and should also be examined by a doctor. Most people affected by this disease also suffer from hair loss. As a rule, Sézary syndrome can be diagnosed by a dermatologist or a general practitioner. Further treatment depends on the exact symptoms. As a rule, this disease does not reduce the life expectancy of the person affected.
Treatment & Therapy
The cure rate for Sézary syndrome is around 50 percent. A commonly used treatment method is photochemotherapy, also known as psoralen plus UV-A (PUVA). The PUVA combines irradiation with UV-A light with the application of psoralen. Psoralen is a group of natural substances. They are found in essential oils and are of plant origin. In PUVA, the psoralen have the task of sensitizing the skin to light.
For this purpose, patients take the psoralen by mouth or have it applied to the area of skin that needs treatment. Creams or water solutions that contain the psoralen can also be used. Depending on which form of PUVA is used, the interval between the administration of psoralen and radiation can vary. The aim of PUVA is to limit the spread of the abnormal cells and possibly even suppress them completely. Treatment with retinoids has the same goal.
These are substances that are similar to vitamin A. Medicine uses retinoids as a means of chemotherapy and applies them primarily when Sézary syndrome is already at an advanced stage. Interferons may also be used to treat the disease. On the one hand, they should inhibit the growth of the abnormal T cells and, on the other hand, activate killer cells that occur naturally in the human body.
The prevention of Sézary syndrome mainly includes general recommendations: healthy nutrition, avoidance of potentially carcinogenic substances and a healthy lifestyle. The exact developmental conditions of the disease have not yet been conclusively clarified; this makes targeted prevention more difficult. However, more recent evidence provides evidence that dietary iron may also be relevant to Sézary syndrome.
Genetic causes are also up for debate: those affected by Sézary syndrome appear to be missing certain gene sequences. In addition, the research was able to find indications of a lack of tumor suppressors, which normally cause cell growth to stop.
The life-threatening Sézary syndrome requires comprehensive therapy with subsequent aftercare. The latter aims to avoid the carcinogens as far as possible. That is why it is extremely important for patients to follow the doctor’s recommendations. After medical treatment, it is helpful to live consciously and eat a balanced diet.
Although there is still no conclusive knowledge about the causes of the syndrome, a health-conscious diet is helpful as a preventive measure and aftercare. Those affected can also take advantage of genetic counseling. The recovery process can be supported by general aftercare measures.
This includes sensible skin care that reduces the redness and itching caused by the disease. Changes to the nails can also be avoided or at least reduced with the help of a balanced diet. If weight loss, fever, or other complications occur following medical therapy, immediate medical evaluation is required.
Sufferers should watch out for such symptoms to improve their chances of recovery. Patients can not only turn to their doctor for detailed advice. Those affected also receive comprehensive information and recommendations for treatment and aftercare at the Max Delbrück Center in Berlin.
You can do that yourself
Sézary syndrome is primarily treated with medication. The patient is prescribed MTX, prednisone and similar drugs that alleviate the symptoms and improve the complexion. Various general measures support the recovery process. Good skin care will relieve itching and redness. Nail changes can be reduced with a healthy diet. The symptoms of the lymph must be treated by a doctor in any case, as various complications such as weight loss or fever can occur.
Sézary syndrome is a life-threatening condition with an overall poor chance of recovery. This makes it all the more important to have comprehensive advice from a specialist who will show you ways and means of improving your quality of life and improving your prognosis. Measures such as chemotherapy or operations should be discussed with the doctor, since the chances of recovery are better the earlier the therapy is started.
The most important self-help measure is to identify the medical options and use them to the full with the support of a medical specialist. The Max Delbrück Center for Molecular Medicine in Berlin-Buch is one of the most important institutes for Sézary syndrome research. Sick people turn to the center for the best possible advice and treatment.