Sézary syndrome is a rare form of lymphoma, a cancer of the lymphatic system. It is characterized by severe rashes and itching.
What is Sézary Syndrome?
“Sezary syndrome is a rare form of cutaneous T-cell lymphoma, which is a cancer of the lymphatic system, explains Professor Martine Bagot (PU-PH, head of the dermatology department at Saint-Louis hospital. It is characterized by a abnormal accumulation of T lymphocytes, a type of white blood cell, in the skin and blood“It is named after Albert Sézary, the French dermatologist who first described it in 1938.”Treatment depends on the progress of the disease. It includes treatments immunomodulators, photopheresis, targeted monoclonal antibodies or chemotherapies. Some cases may benefit from marrow allograft“. As with many serious illnesses, prognosis and response to treatment vary from patient to patient.
What are the symptoms of Sézary Syndrome?
Symptoms of Sézary syndrome usually include severe rashes and itching. “Patients may present with rashes in the form of red, scaly and sometimes thick patches on the skin, specifies the Professor. These rashes can affect the whole body (erythroderma)“. Itchy skin (pruritus) are often one of the most common symptoms annoying and disabling Sézary syndrome. “They can be extremely intense and alter the patient’s quality of life“. When the patient scratches a lot, the lymph nodes have an increased size and are palpable, especially around the neck, armpits and groin. “In some patients, we can also see hair loss” as well as a depigmentation or a hyperpigmentation skin.
What is the cause of Sézary Syndrome?
The precise cause of Sézary syndrome is not known. “However, studies have eliminated several causes. The abnormal accumulation of T lymphocytes is not not linked to a viral or genetic cause, environmental origin (exposure to chemicals or carcinogens) is not demonstrated“, continues the specialist. Research is still ongoing to better understand the mechanisms of the disease. “It is important to note that Sézary syndrome is a rare disease. It only represents 5% of all cutaneous lymphomaswhich are themselves very rare“. It affects men and women in an almost equal manner, aged one sixty years.
What is the life expectancy?
Life expectancy for people with Sézary syndrome varies depending on many factors, including the stage of the disease at diagnosis, response to treatment, tolerance to treatments, age and health status. general condition of the patient. “But overall the prognosis is much better than before when it was only 5 years, assures the Professor. Today, thanks to the development of new targeted therapies, the outlook is clearly improved.”
The diagnosis of Sézary syndrome is generally made by a doctor specializing in dermatology or in hematology/oncology and requires a series of thorough examinations including skin biopsies and blood tests.
Symptoms may resemble those of other skin and lymphatic conditions.
“Because the symptoms of Sézary syndrome may resemble those of other skin and lymphatic conditions, an accurate diagnosis is essential to determine the nature of the disease.“, supports Professor Bagot. Often consulted as first intention, the attending physician will refer to a dermatologist. “The latter will carry out a skin biopsy (taking a sample of affected skin for microscopic examination in the laboratory). In 70% of cases, the biopsy determines the presence of abnormal T lymphocytes. in the skin, which is characteristic of Sézary syndrome“. If the biopsy does not provide a diagnosis, the patient should be referred to a center of the INCa cutaneous lymphoma network for more in-depth examinations. “Of the molecular biology techniques, such as analysis of T receptor gene rearrangements or flow cytometry, can be used to detect a dominant T clone or an abnormal distribution of lymphocyte subpopulations.s”. Once the diagnosis of Sézary syndrome is confirmed, the doctor can discuss appropriate treatment options based on the severity of the disease and the patient’s overall health. As it is a disease complex, the management of Sézary syndrome generally requires a multidisciplinary medical team specializing in dermatology, hematology/oncology and pathology.
Can it be cured and with what treatment?
Sézary syndrome is a rare and complex disease. Its management is generally carried out by a medical team specializing in dermatology, hematology/oncology and pathology. “Treatment is generally based on a combination of a systemic and local approach“, explains the specialist.
► The first step is the photopheresis. “A volume of blood is taken from the patient. Blood is processed to separate blood cells, including T lymphocytes, from other blood components, such as red blood cells and platelets. The T lymphocytes are then exposed to a photosensitizing drug and specific ultraviolet A (UVA) rays. This step aims to modify tumor T lymphocytes and stimulate anti-tumor responses. After exposure to UVA, blood cells, including modified T lymphocytes, are reinfused into the patient.”.
► Photopheresis is supposed to act by modulating the immune response by reducing the population of pathogenic T lymphocytes, by making them less reactive, and increasing immune tolerance. “The procedure is repeated every 15 days for 6 months to 1 year depending on the patient’s response to treatment.
► Photopheresis can be used alone or in combination with other therapiessuch as oral or injectable immunomodulators.
► The monoclonal antibodies constitute a real therapeutic breakthrough in the treatment of Sézary syndrome. “Certain targeted therapies, such as mogamulizumab, a monoclonal antibody targeting CCR4-positive T cells, have proven effective on the proliferation of skin cells. In addition, side effects are more limited than for chemotherapy.“. Marrow transplantation is another treatment option.
Carried out by a hematologist, it is aimed at patients under 70 years of age, without comorbidities (diabetes, renal or heart failure). “If transplantation is not possible, several chemotherapy programs can be offered.” Some people with Sézary syndrome achieve complete or partial remission in response to treatment(s). “Unfortunately some patients usually die due to infection linked to a decrease in “normal” lymphocytes“.
Thanks to Professor Martine Bagot, PU-PH at Saint-Louis Hospital (AP-HP) and Paris Cité University.