The diagnosis is complex because there are several subtypes and the symptoms are different, often confused with those of other skin diseases. So it takes patients up to three or four years before they know what they have
It is a little-known tumor with a complex diagnosis because there are numerous subtypes with different characteristics and variable symptoms, often very similar to those of common skin diseases. Thus those approximately 500 Italians who are diagnosed with cutaneous lymphoma every year have to deal with a series of additional problems, because symptoms and signs may be misleading at first and it can take a long time (even three or four years) before to meet with an experienced specialist who can to arrive at an accurate diagnosis.
Patient identification
Cutaneous lymphoma it is part of non-Hodgkin’s lymphomas a cancer of the lymphatic system that starts in the skin from cells of the immune system, called lymphocytes. It is actually a heterogeneous group of diseases of the lymphatic system involving lymphocytes, with the skin as the organ involved at onset and, often, as the only organ affected. It is not at all easy to classify them in a precise way because there are different variants and moreover they are not very frequent tumors. It strikes especially people over 50 and overall more common in men than women, although gender prevalence varies in different subtypes Nicola PimpinelliProfessor of Dermatology at the University of Florence, one of the leading Italian experts in this pathology.
What are the symptoms
The initial symptoms and signs are variable depending on the subtypes and can change over time — continues Pimpinelli, who with other expert colleagues coordinates clinical research on cutaneous lymphoma within both the Italian Society of Dermatology and Venereology (SIDeMaST) of both national and international multidisciplinary groups —. Basically there are cases that start as superficial spots and blotches, reddened and sometimes scaly, accompanied by intense itching, which over the course of months or even years can extend to all (or almost all) of the body surface or thicken until they become plaques or nodules or even large tumors. While in other cases the initial lesions are already plaques or nodules of variable sizewhich in a shorter or longer time grow to become large nodules or tumors.
Because of the diagnostic delay, then, sufferers often suffer from sleep problems, resulting in chronic fatigueand depression due to discomfort caused by both skin disorders and intense itching.
How is the diagnosis made?
It often happens that, at least initially, the skin lesions are mistaken for a different skin disease, such as psoriasis and dermatitis, or from an infection. To arrive at a well-defined diagnosis, you need a thorough visit with a dermatologist specialist, who will decide how to proceed. The biopsy is the fundamental examination (a small sample of skin from the suspect region is taken and analyzed under a microscope, also in order to identify specific markers present on the membrane of the cancer cells or any changes in genes and chromosomes) to identify the subtype; you must then proceed with further investigations (blood test, CT scan, Pet and possible bone marrow biopsy) to understand if the disease eventually extended to the lymph nodes and internal organs.
What are the subtypes
The first subdivision of cutaneous lymphomas is based on the identification of the type of lymphocytes involved in the growth of the tumor – explains Pimpinelli -: in fact there are cutaneous T-cell lymphomasinvolving T lymphocytes (immune cells that defend the body against microorganisms by attacking them directly or by producing substances that help defeat them) and cutaneous B-cell lymphomas, the immune cells responsible for producing antibodies. 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 transform into sindrome di Gray
(rarer but more aggressive), which is actually a distinct disease and can develop and present independently of mycosis fungoides.
Treatment available
The goal of treatment is to relieve symptoms such as pain, itching, burning and redness, to control the disease and to provide the best possible quality of life. Thanks to medical-scientific research new therapies for cutaneous lymphomas are now available and have been shown to delay the progression of the disease. essential, to arrive at a precise diagnosis and establish the most suitable therapy for the individual patient, a multidisciplinary work between dermatologist and hematologist.
The methods of treating cutaneous lymphoma vary according to the subtypes and stage of the disease, if only the skin is affected or if there is already blood or lymph node involvement – concludes the expert -. The treatments are substantially distinguishable in therapies aimed at the skin (phototherapy, topical chemotherapy), radiotherapy and systemic therapies (biological response modifiers, chemotherapy, immunotherapy, immuno-chemotherapy). Furthermore, for patients with high-risk disease, it may be necessary to use the stem cell transplant. To date, in patients who regress the goal is not only remission but also obtaining a stable response over time.
March 10, 2023 (change March 10, 2023 | 09:42)
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