Myeloid leukemia: what it is, with what symptoms it manifests itself and what is the difference between acute and chronic form

After the premature disappearance in 2022 of the Serbian coach Sinisa Mihajlovicaffected by acute myeloid leukemia, a new case now shakes the world of sport: a form of Myeloid leukemia It was diagnosed with the basketball player Achille Polonara33 years old, wing of Virtus Bologna, who already passed had also had to face testicles cancer. The athlete is currently hospitalized at the Sant’Orsola Malpighi hospital in Bologna, where he started specific therapies.

Achille Polonara, 33 years old, wing of Virtus Bologna received a diagnosis of myeloid leukemia

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In Italy, about 9,000 patients affected by this rare tumor of the blood are which has a strong impact on the quality of life, as reported also by the recent research «The quality of life of patients with LMC», Promoted by Novartis Italia and created by Elma Research, which highlighted the need to also priorize the needs related to daily management and long -term quality of life. There Chronic myeloid leukemiaIn particular, it represents 15-20% of all cases of leukemia, with an incidence of 1-2 cases per 100,000 individuals per year. It mainly affects adults and has a progressive course.

Chronic myeloid leukemia, the causes

To cause the Chronic myeloid leukemia (LMC) is the uncontrolled proliferation of the stem cells of the bone marrow.
In particular, in the LMC, a specific genetic alteration leads to the formation of an anomalous chromosome, called Philadelphia chromosome. This anomaly occurs when a part of chromosome 9 merges with chromosome 22 and vice versa. This fusion creates a new anomalous gene called BCR :: ABL1. In the case of LMC, the BCR :: ABL1 gene changes the behavior of bone marrow cells, causing the growing growth and proliferation of leukemic cells, which do not respond to the normal mechanisms of growth and cellular vitality: they live longer, grow more and are more unstable.

The natural course of the LMC is characterized by a biphasic and sometimes three -phase trend: chronic phase, accelerated phase and blastica phase. The LMC is diagnosed in the chronic phase in most patients (80%). About half of the patients are asymptomatic or has slight symptoms. Patients with Diagnosis of LMC in the chronic phase if treated have an expectation of normal or almost normal life expectancy.

The symptoms of chronic myeloid leukemia

About half of patients with LMC is asymptomatic and discovers the disease only Following a routine bloodocromo that highlights a high count of white blood cells. When present, the symptoms are often mild and non -specific, making it difficult for an early diagnosis. There persistent tirednessThe general malaisethe Loss of appetite and one feeling of early satiety they are common and can be linked toanemia and toenlargement of the spleen (SPARTOMEGALY), which can also cause abdominal pain.
With the progression of the disease towards the accelerated or blastica phase, the symptoms become more severe. A worsening of anemia are observed, intense bone pains, persistent fever and a increased risk of serious infections or spontaneous bleeding.

The prospects of care

The objective of the treatment of the LMC, both for the chronic phase and for the accelerated phase, is the same: to eliminate all the cells that contain the BCR :: ABL1 gene, leading to the remission of the disease. The main feature of the LMC is a particular chromosomal alteration: a translocation that produces a protein called tyrosine Chinase BCR-ABL110, always active and responsible for the anomalous growth of the cells. The discovery of the molecular target in the LMC has made it possible to develop specific drugs: tyrosine inhibitors Chinase (TKI).

Tki drugs aim to block the Chinasi Chinasi BCR-ABL1, representing enormous progress for patients with LMC, allowing a long survival. However, most patients should continue therapy for life. Therefore, it is important that the treatment is not only effective, but also well tolerated.

Despite these therapeutic progress, some patients do not respond to treatment or lose the response or manifest signs and symptoms of treatment intolerance. In these cases it is necessary to stop the therapy chosen in the front line and move on to a different inhibitor (second line therapy). Today, even for resistant and/or intolerant patients at the second line of therapy there are treatment prospects.

52% of second -line patients carry out the other drug. In the third line there are effective therapies such as Asciminib, which is reimbursed in Italy for patients with chronic myeloid leukemia, with resistance or intolerance to at least two previous tyrosine-chinasic inhibitors.

Acute myeloid leukemia, the most aggressive

The former Bologna coach Sinisa Mihajlovic who died at 53 years due to acute myeloid leukemia

The former Bologna coach Sinisa Mihajlovic, who passed away at 53 years due to acute myeloid leukemia

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There Acute myeloid leukemiaUnlike the chronic form, it is an extremely aggressive pathology of leukemia that most likely affects men over 60 years, although it can also arise in children.

According to the data of theItalian Association of Tumor Registers (Airtum) reported by AIRCevery year in Italy just over 2,000 new cases can be estimated: 1,200 between men and 900 among women.

It is the tumor form that the Serbian coach suffered Sinisa Mihajlovic, for which he had also undergone a bone marrow transplant.

With what symptoms it manifests itself

The symptoms of the Acute myeloid leukemia They manifest themselves early and usually the diagnosis is made shortly after their appearance. Often they are non -specific symptoms, such as tiredness, Loss of appetite, night sweating, fever. Later they can manifest themselves:

-Sposty and pallor linked to anemia,
increased risk of infections due to the reduction of normal white blood cells
-Sanguinations frequent (also at the gingival or nasal level) linked to the deficiency of platelets
-falling muscle pain And widespread osteo-articular
General sense of malaise
– weight loss

If the disease has spread to other organs, a Milza enlargement, liver And lymph nodes and, if the nervous system has also been reached, they could present themselves heachache And Other neurological signs.

Possible therapy and what the search is doing

At present, The most effective therapy for many patients with acute myeloid leukemia is donor marrow transplantation. The anticancer activity of the transplant is due to the partial incompatibility between the donor’s immune system and the patient’s cells, which also includes cancer cells. This mutual incompatibility is linked to the presence, on the surface of the cancer cells, of a different class of protein, called HLA. This diversity facilitates the work of the donor’s lymphocytes in recognizing the tumor as a foreign element, to be attacked and eliminated.

Currently, a new research and development project of innovative therapies for the treatment of recurrence acute myeloid leukemia or conventional care resistant – financed by Veronesi Foundationwith theBambino Gesù Pediatric Hospital of Rome as a reference center, the centralized diagnostic laboratory of the Oncohematological clinic of Paduathe Experimental Oncology Department ofEuropean Institute of Oncology of Milan and the Department of Leukaemia dell ‘MD Anderson Cancer Center (Houston USA) as support centers – is concerning clinical experimentation, the first in the European field, of Gene therapy with Car-Natural Killer cells.

Source: Vanity Fair

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