Michele Merlo, a former competitor of Amici and XFactor, died at the age of 28 from a cerebral hemorrhage triggered by fulminant leukemia. What is it about? Acute promyelocytic leukemia, better known by the expression of fulminant leukemia, is a subtype of acute myeloid leukemia and is the most aggressive form of blood cancer. If not diagnosed in time, this disease, which in Italy affects about 150 people a year, especially between the ages of 30 and 40, can lead to death in a few hours or days. This is why what makes the difference in these cases is the possibility of promptly arriving at a correct diagnosis.
Fulminant leukemia: what is the cause?
There is one underlying cause of fulminant leukemia acquired translocation, that is, an unusual change in the shape of chromosomes, with the movement of a piece from one chromosome to another. This alteration, not present at birth, occurs between the chromosomes 15 and 17. Even today, however, the mechanism at the origin of the modification at the level of the two chromosomes is not known. This process, then, involves a reduction in the number of platelets and an alteration of the mechanisms of coagulation.
What are the symptoms?
The disease begins and progresses rapidly, so the onset of symptoms is early. Indeed, fulminant leukemia manifests itself with skin bleeding, visible in the form of unexplained dark bruises, bleeding from both nostrils, from the gums, from the digestive or genitourinary system. There can also be rather nonspecific ailments, such as fatigue, fever, loss of appetite, excessive sweating, but also a increased risk of infections, which can result in sore throat and swollen lymph nodes, headache and other neurological signs. Furthermore, from the latest estimates, it emerged that 10-20% of patients undergo fatal major hemorrhages, for example in the brain, even before they can receive the diagnosis and therefore undergo treatment.
How is fulminant leukemia diagnosed?
If recognized in time, fulminant leukemia can be treated effectively but, in fact, it is necessary to reach a diagnosis as soon as possible, which can be formulated through a morphological examination of the bone marrow and peripheral blood. To get a more complete picture of the situation, we always tend to look for the typical cytogenetic and molecular anomalies, fundamental characteristics of this disease. Since the origin of the disease is a chromosomal translocation, international guidelines recommend performing the genetic diagnosis, which consists in identifying, through cytogenetics and molecular biology techniques, the alteration of chromosomes 15 and 17. This evaluation also allows patients to be stratified on the basis of risk percentages. In this way it is possible to modulate the therapy to be undertaken after the remission of the disease.
How is it treated?
Unlike fifty years ago, fulminant leukemia can be cured today. The merit is above all of a group of Italian hematologists who, in 2013, published a very important study on New England Journal of Medicine. Research has greatly reduced the role of chemotherapeutic agents and, at the same time, has demonstrated the effectiveness of a new therapeutic approach based on combination of retinoic acid (derivative of vitamin A) with arsenic trioxide. The latter, in fact, induces the programmed cell death of cancer cells, with the help of retinoic acid. The disease can also be treated with transfusions of platelet concentrates, fresh frozen plasma and blood products. In 20-25% of cases, however, this type of leukemia tends to relapse, therefore these patients can benefit from a hematopoietic stem cell transplantation (autologous or allogeneic).