2023-10-29 11:43:41
“I have leukemia, now I have the courage to say it.” Wanda Nara revealed yesterday, through a post published on social media, the name of the disease against which the showgirl and wife of striker Mauro Icardi has been fighting for some time. But what is leukemia, what are the symptoms and what are the treatments? The San Raffaele experts explain it in a focus drawn up on the occasion of the National Day for the fight against Leukemia, with the answers of Professor Fabio Ciceri, head of Hematology and Bone Marrow Transplant at the IRCCS San Raffaele Hospital.
What is leukemia?
When we talk about leukemia – explain the experts – we are not talking about a single disease, but about a set of blood tumors caused by the uncontrolled proliferation of hematopoietic stem cells, called leukemia or blasts, i.e. those still immature cells which then grow and give rise to the components of our blood: white blood cells, red blood cells and platelets.
Blood stem cells originate in the bone marrow, present in adults especially in the flat bones (such as the pelvis, sternum, skull, ribs, vertebrae, shoulder blades) and can follow 2 lines of development:
– the cells of the myeloid lineage give rise to white blood cells (in particular neutrophils, monocytes), platelets and red blood cells;
– the cells of the lymphoid line give rise to white blood cells, also called lymphocytes.
“Following genetic mutations and complex mechanisms, which are not always fully understood today, stem cells can prematurely interrupt their development, or they can acquire the ability to replicate without limit and become resistant to programmed cell death mechanisms (apoptosis). When this happens, the immature cells invade the marrow, blood and sometimes the lymph nodes, spleen and liver and thus give rise to leukemia”, explains Professor Ciceri.
The different types of leukemia, data on the disease
“One of the classification factors of the disease is its speed of progression: the acute forms have a short or very short evolution and also present a block in the maturation of the cells, while the chronic forms have a slower evolution, in which However, the ability of the marrow precursors to mature is maintained, although it may be abnormal”, clarifies the hematologist.
According to the National Cancer Registry, the most frequent forms are:
chronic lymphatic (33.5% of total leukemias);
acute myeloid (26.4%);
chronic myeloid (14.1%);
acute lymphatic (9.5%).
Tumors that affect blood cells are much more frequent in childhood than in adulthood. In particular, acute lymphoblastic leukemia represents 75% of all leukemias diagnosed in children up to 14 years of age, while acute myeloid leukemia represents 15-20%.
In adults, however, the majority of acute leukemias are represented by myeloid forms, while lymphoblastic forms are less frequent, around 25-30%. Chronic leukemias are instead typical of adulthood, while they are rare in pediatric age.
Based on AIRTUM data (Italian Association of Cancer Registries), we find the:
– B-cell chronic lymphocytic leukemia (30% of all leukemias);
– acute lymphoblastic leukemia (20% of leukemic forms of advanced age);
– chronic myeloid leukemia (1-2 cases per year per 100,000 adults)
– acute myeloid leukemia (3.5 cases per year per 100,000 adults).
Although the incidence of leukemia is growing, with almost 8,000 new cases diagnosed in Italy in 2022, mortality is decreasing thanks to a continuous and constant improvement in therapies.
What are the symptoms
In the initial stages, experts continue, chronic leukemias may not cause symptoms because the leukemia cells interfere in a limited way with the functions of other cells. Unlike acute leukemia, symptoms appear early and can worsen extremely rapidly.
“Fever, night sweats, tiredness and fatigue, headache, bone and joint pain, weight loss, pallor, are usually associated with a lack of red blood cells, therefore with a condition of significant anemia. In the case of platelet deficiency, they may appear even mild hemorrhages in the oral cavity or gastrointestinal tract or skin spots. In a subsequent step, but always very rapid, deeper hemorrhages in the brain or at the level of the gastrointestinal tract can also appear”, explains Ciceri.
“While in the case of proliferation of white blood cells – he adds – there may be symptoms similar to a bad flu syndrome, such as continuous, but well tolerated fever. Sometimes the leukemic blasts can also infiltrate organs such as the stomach, intestines, kidneys, the lungs or the nervous system, giving specific symptoms that indicate poor functioning of the organ involved”, concludes the professor.
The cure
Over the last few years, explains San Raffaele, there has been a constant and progressive improvement in the prognosis of the disease for 2 reasons: we know better the factors underlying these diseases, in particular the genetic mutations associated with the more aggressive forms, and therefore we can proceed with a transplant in the early stages, when the patient is less compromised, has undergone fewer therapies and better results can be obtained; and a constant improvement of therapies that are combined based on the characteristics of the individual patient’s disease.
Hematopoietic stem cell transplantation:
Hematopoietic stem cell transplantation is one of the therapeutic options in use to replace diseased cells, destroyed with high doses of chemo or radiotherapy, with healthy cells from a compatible donor. Often the donor is a sibling or family member, but it can also be a stranger who has cells compatible with those of the patient.
Professor Ciceri states: “In some cases this approach is able to definitively cure leukemia, especially in younger patients, and can be used for forms that no longer respond to chemotherapy. This is why it is important to sign up to the bone marrow donor register, a simple gesture that allows, through the sampling of a small quantity of blood or saliva for genetic typing, to save a life, even in the future.”
Chemotherapy and immunotherapy with CAR-T cells:
In addition to transplantation, the other therapies available today are chemotherapy combined with other approaches, aimed at stimulating the immune system to recognize and destroy leukemia cells, such as, for example, interferon alpha used to slow down the growth of tumor cells, or monoclonal antibodies capable of targeting leukemic cells, favoring their destruction by the immune system.
“An innovative therapeutic approach in recent years is immunotherapy with CAR-T cells, an option available for some leukemias that do not respond to conventional treatments. CAR-T cells are the patient’s own T lymphocytes which are genetically modified so as to be equipped with the CAR molecule (chimeric antigen receptor). Thanks to this molecule, once they are reintroduced into the patient, CAR-T cells are able to specifically recognize tumor cells and effectively attack and destroy them. Leukemia is the first tumor treated with this therapeutic tool which has been available in Italy since 2019″, explains the professor.
In Italy, the first patient with CAR-T cells was infused at the San Raffaele Hospital, coordinating center of the international ELARA clinical trial. The expertise of doctors and researchers has also been fundamental for the development, from the laboratory to the first phases in the clinic, of other similar experimental treatments such as CAR-T therapies for multiple myeloma and acute myeloid leukemia.
“These ‘tailored’ approaches, built on the evolution of the disease and on the possibility of combining and dosing multiple treatments, have allowed us to achieve not only long-term survival objectives, but also great improvements in the quality of life of our patients” , concludes the professor.
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