There are about 500 new cases a year in Italy. Mycosis fungoides and Szary syndrome are the two main types of cutaneous T-cell lymphomas. Long time to diagnosis, but treatments can relieve symptoms
a very rare and somewhat strange form of cancer, which considerably complicates the life of those who suffer from it, so much so that it can take several years to arrive at a certain diagnosis. Cutaneous lymphoma is a cancer of the lymphatic system that originates in the skin, starting with immune system cells called lymphocytes. Thus, what makes this neoplasm peculiar and more difficult to detect is the fact that the symptoms are very similar to those of other more common skin diseases, such as eczema and psoriasis: reddish patches often associated with itching, a lump that may also appear under the skin or an ulcer, which does not go away.
500 new cases per year in Italy
Skin lymphomas belong to the group of non-Hodgkin’s lymphomas – he explains Giuseppe Argenziano, full professor and director of the Dermatological Clinic of the University of Campania Luigi Vanvitelli in Naples -. I am a heterogeneous group of diseases of the lymphatic system involving lymphocytes, with the skin as the organ affected at the onset and, often, as the only affected organ. It is not at all easy to classify them precisely because there are several variants and moreover they are not very frequent tumors. The first subdivision is however based on the identification of the type of lymphocytes involved: they exist in fact cutaneous T-cell lymphomas (involving T lymphocytes, cells that defend the body from microorganisms by attacking them directly or by producing substances such as cytokines that help defeat them) and cutaneous B-cell lymphomas, those responsible for the production of antibodies. There are about 500 new diagnoses every year in Italy, mainly concern men and appear mostly in the age group between 55 and 60 years.
Cutaneous T-cell lymphoma
Cutaneous T-cell lymphoma is the most common type and typically occurs with red, scaly patches or plaques on the skin that appear in areas of the body usually not exposed to the sun. They can also involve the blood, lymph nodes and some internal organs. 80% of people report having itching: it often looks like eczema, psoriasis or other chronic dermatitis and this is the reason why the diagnosis is delayed – explains Argenziano -. Only a minority of patients develop advanced disease: Most people have indolent (ie chronic, slow-growing) lymphomas, but getting a timely diagnosis is essential to start treatment right away and prevent the situation from worsening. Mycosis fungoides, the best known type of T-cell lymphoma, is also the most common form of cutaneous lymphoma. In some cases the disease can evolve and turn into Szary syndrome (rarer but more aggressive), which is actually a distinct disease and can develop and present independently of mycosis fungoides.
Mycosis fungoides
Mycosis fungoides begins with particular skin spots (located mainly on the buttocks and on other areas not exposed to the sun such as the lower part of the trunk, thighs, breasts), which itch and slowly progress to result in infiltrated plaques or tumors. The lymph nodes are the extracutaneous site most affected, but organs (liver, lung, spinal cord) may also be involved. The disease must be treated by a multidisciplinary team as in the initial forms the dermatologist who makes the diagnosis and prescribes the therapy – says the expert -. For the more advanced and important forms it is necessary the close collaboration of a haematologist and other specialists. Centers of reference are therefore needed to ensure adequate examinations and treatments for people facing a very difficult pathology. In fact, only half of all patients with live advanced stage lymphoma five years after the onset of the disease. It should not be forgotten that both mycosis and Szary’s syndrome are characterized by a progressive physical disfigurement, uncontrollable itching, pain, lymphadenopathy and alopecia: all of this has a negative effect on the quality of life of patients, as well as on the emotional and social well-being of their families.
Specific tests to arrive at the diagnosis
What if the biopsy represents the most useful examination to obtain a certain diagnosis of cutaneous lymphoma (a small sample of skin from the suspected region is taken and analyzed under a microscope), to then understand which subtype it is, it is necessary to proceed with further investigations (blood analysis, CT scan, Pet and magnetic resonance) that allow, for example, to identify specific markers present on the membrane of tumor cells or any changes in genes and chromosomes. Accurate clinical and instrumental staging are essential to prescribe the most suitable treatments and keep the disease under control – he stresses Marco Ardig, Head of the Rare Diseases and Porphyrias Operating Unit of the IFO-San Gallicano in Rome -. To manage this broad spectrum of rare neoplastic diseases it is necessary an early diagnosis, obtainable only with the standardization of diagnostic procedures. The use of personalized therapies, considering the chronicity and long duration of the common forms, but also the aggressiveness of the rare forms.
The therapies
The goal of treatment relieve symptoms such as pain, itching, burning and redness, control the disease and provide the best possible quality of life. Thanks to medical-scientific research they are now available new therapies for cutaneous lymphomas. They have shown that they can delay the progression of the disease – adds Argenziano -. At the same time they improve the quality of life of patients e they are usually well tolerated. It is very important to be able to detect cutaneous T-cell lymphoma as soon as possible in order to have a better chance of being able to stop the disease. There are many factors that influence the choice of the most suitable treatment (the subtype of cutaneous lymphoma, the type and extent of lesions on the skin, the involvement of the lymph nodes or other organs), but the most important the clinical stage of the disease. Based on various parameters, treatment options may include topical creams or ointments, light therapy, oral medications or infusion (intravenous) therapies, chemotherapy, and radiation therapy – concludes Ardig -. In patients with mycosis fungoides in the early stages the therapy is usually cutaneous, while in the advanced stage it is often proceeded with systemic therapies, which are also used for Szary’s syndrome. In addition, for patients with high-risk disease, it may be necessary to resort to allogeneic blood stem cell transplantation. In recent years, new drugs have arrived (including monoclonal antibodies such as mogamulizumab and brentuximab vedotin, now also available in Italy) and several other molecules are currently being tested.
Who at risk
The causes that lead to the development of cutaneous lymphoma are not yet fully known. Certainly, an immune system that is not functioning at its best poses a risk: for this reason the likelihood of having cutaneous lymphoma is higher in people with immunodeficiency syndromes, such as AIDS, or people previously dealt with chemotherapy for other cancers or with drug-induced immunosuppression states, or in people who have undergone a organ transplant and treated with anti-rejection drugs. Some infections can also increase the risk of cutaneous lymphoma, particularly those from HIV (the virus that causes AIDS) or other rare viruses such as HTLV-1 that cause particular forms of cutaneous lymphoma.
December 6, 2021 (change December 6, 2021 | 18:20)
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