The news that is bouncing around the media concerns the French actor Alain Delon, who according to what is reported has a “slowly evolving lymphoma” and has expressed the desire to resort to euthanasia. Obviously nothing can be said about the specific case, for which there is no verified information from the doctors treating him: neither the form of lymphoma he is suffering from, nor the therapies followed, nor his general health conditions are certain. of Delon, who had already had a stroke in 2019. We therefore asked the experts from the IRST ‘Dino Amadori’ IRCCS of Meldola to explain in general what this blood cancer is, how many types there are and how they are treated today.
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What are “slowly evolving” lymphomas
Lymphomas are blood cancers caused by the uncontrolled growth of an immune system cell, called a lymphocyte, and usually develop in lymph nodes or lymphatic organs, such as the spleen. They are divided into two large groups: Hodgkin lymphomas (named after the doctor who first described them) and non-Hodgkin lymphomas. The latter, in turn, can be distinguished into “aggressive” and “indolent” tumors, as could be the one that affected Delon and which normally manifest themselves in advanced age. “In reality, this division based on aggression is very crude,” he explains to Oncoline Gerardo Musuraca, director of the Simple Hematology and Transplant Structure of the IRCCS IRST Dino Amadori of Meldola – These are tumors that evolve very slowly and have a clinical course characterized by remissions and relapses. Among these we find follicular lymphoma – which is the most frequent -, marginal zone lymphoma, small lymphocyte (or lymphocytic) lymphoma and lymphoplasmacytic lymphoma (or Waldenstrom’s disease). In the past, mantle cell lymphoma was also considered indolent, but in reality it can have both indolent and aggressive characteristics.”
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Symptoms of indolent lymphomas
Precisely because these lymphomas grow very slowly and often do not cause symptoms, in most cases they are not diagnosed until they reach large dimensions. The most typical sign is the enlargement of one or more lymph nodes, which, however, is only noticed if it occurs at a superficial level. If, however, it is located deep down, explains Morecchio, for example in the abdomen, the lymph node can continue to grow for years, until it reaches considerable dimensions, of 10-20 centimeters. “In these cases, lymphoma is usually discovered by chance through a follow-up exam, which is why it can be useful to have an ultrasound of the abdomen every year – continues Morecchio -. Or a clinical symptom may appear when the lymph node compresses a blood vessel or bile tube, resulting in swelling of a limb or jaundice, for example. Another symptom may be the enlargement of the spleen, more frequent especially in marginal lymphoma, which in this case is called ‘splenic’. Blood tests, however, can rarely reveal the disease: it happens in lymphoplasmacytic lymphoma, where there is an exaggerated production of the IGM antibody.”
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The treatments
Typically, these types of indolent lymphomas easily go into complete remission, but they also have a tendency to continually relapse, and are therefore very difficult to cure permanently. Much depends on the type of lymphoma, the extent and the patient’s condition, but survival from the moment of diagnosis can reach 15-20 years. “We are therefore talking about long-term survivors, and in recent years new therapies have arrived which are aiming both at further prolonging the remission period and at definitive recovery – continues the hematologist -. Combinations of immunotherapies and chemotherapies are used in the first line, while experimental therapies are approaching for the second line. There is a lot of excitement, especially regarding immunotherapy. Furthermore, for follicular lymphoma, two cell therapies have recently been approved for the third line of treatment in Italy: a CAR-T therapy and a bispecific antibody, which appear to be able to bring even patients into complete remission for a very long period of time. who have many relapses behind them.”
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The new therapies are well tolerated even by elderly patients, but it must also be said that immediate treatments are not always necessary: ”We must always evaluate on a case-by-case basis – he adds Giovanni Martinelli, Scientific Director of the IRST – In some patients the best approach is the “watch and wait” one, i.e. watch and wait, and there are series of schemes adapted for the elderly”. Then there are cases in which indolent lymphomas transform into aggressive ones, but this implies a complete change in their nature and, consequently, a different treatment.
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Know the molecular profile of the tumor
From the news, it is not clear whether Alain Delon, who is now 88 years old, has undergone an experimental therapy or whether he is now undergoing palliative treatment. “Always speaking in general – given that we know nothing about the actor’s clinical history – we can say that today we manage to treat haematological tumors well even in patients over 90 years of age and with a high probability of success – he says Claudio Cerchione, Medical Research Director of the Hematology Division – It is essential to always carry out correct staging through imaging techniques, considering that tumors of the blood and lymphoid organs are systemic by definition, therefore the concept of metastasis does not exist. Furthermore – he concludes – the genetic-molecular characterization of the disease is increasingly important, on which the choice of treatment depends”.