a subtype of non-Hodgkin lymphoma that affects mostly people over 70 and usually progresses slowly. The innovative drug is better tolerated by patients
The macroglobulinemia di Waldenstrm it takes its name from the Swedish doctor who first described it in 1944, observing two patients who had anemia, bleeding, enlarged lymph nodes, the presence of abnormal amounts of plasma cells in the bone marrow and an increase in blood viscosity. been little known for a long time, but has reaped illustrious victims which have earned it the name of disease of princesbecause it resulted in the death of the French president George Pompidou and of the last Shah of Persia, Reza Pahlevi. Today, this blood cancer is less scary: There are several effective therapies and also recently available in Italy a new drug for those patients who have already received at least one previous treatment or, as first-line treatment, for those unsuitable for chemo-immunotherapy.
Vague symptoms
Waldenstrm’s macroglobulinemia a relatively rare lymphoproliferative disorder and usually progresses slowly – explains Marzia Varettonimedical director of the Hematology Division at the IRCCS Policlinico San Matteo di Pavia Foundation —. a subtype of non-Hodgkin lymphoma which mainly affects people over seventy and is mainly located in the bone marrow, although lymph nodes and the spleen may also be involved. The most common initial symptoms are non-specific, similar to those of many other diseases (weakness, loss of appetite, fever, sweating, weight loss and neuropathy). Less frequent signs are swollen lymph nodes, abdominal swelling, bleeding or heart problems. Often, therefore, the diagnosis is made thanks to classic control blood tests. About a quarter of patients, however, are asymptomatic and do not require immediate treatment, but only performs periodic checks. If, on the other hand, the therapies are necessary, we proceed according to the individual case, based on the characteristics of the disease and the age of the patient.
Therapy
In a very small percentage of patients it is necessary to act under emergency conditions sessions of plasmapheresis (plasma subtraction) to reduce blood viscosity due to the accumulation of an abnormal protein. Otherwise, when the disease becomes symptomatic, treatments are started, which are numerous today and which have considerably expanded in recent years thanks to the introduction of target therapies such as BTK inhibitors. These are targeted drugs capable of inhibiting, only in the tumor cell, those signals that favor its survival – clarifies Varettoni -. They are administered orally, with an excellent tolerability profile, rapid and excellent disease control and are able to slow its progression. Among the advantages of this type of therapy is that of making treatment more acceptable in elderly people, perhaps already tried by other pathologies, and limiting admissions to hospital. Zanubrutinibthe new drug that has recently received the green light from the Italian Medicines Agency (Aifa), in fact, an oral Bruton’s tyrosine kinase inhibitor (aka BTK): Approval of zanubrutinib offers an important new treatment option for these patients,” he comments Pier Luigi Zinzani, Professor of Hematology at the University of Bologna, Seragnoli Institute of Hematology -. Aifa’s ok is based on efficacy and safety results from the Phase 3 ASPEN study which compared zanubrutinib to ibrutinib, the first BTK inhibitor to be approved in Waldenstrm’s macroglobulinemia, and demonstrated that the new medicine is a better option for patients. In Italy, patients will therefore have the opportunity to be treated with an innovative molecule which could lead to a deep and lasting response with better tolerability, as emerges from the ASPEN study.
December 14, 2022 (change December 15, 2022 | 11:35 am)
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