For Michele Merlo there was talk of fulminant or hyperacute leukemia, is this the diagnosis?
“It is a somewhat vague term – replies Fabrizio Pane, professor of hematology at the Federico II University of Naples – it was most likely of acute promyilocytic leukemia – a subtype of acute myeloid leukemia – characterized by being frequently complicated by a severe form of “disseminated intravascular coagulation” which in some cases can constitute the clinical picture of the onset of the disease. This type of leukemia is very aggressive precisely because it often appears with a hemorrhage in the brain, as in the case of this unfortunate boy.
What does it depend on?
«Like all leukemias, it is a bone marrow disease, which is the production site for all blood cells including white blood cells. As a result of the leukemic transformation, these remain blocked at an immature stage and do not “work”, without however losing their replicative capacity. They therefore replace the normal blood cells of the marrow preventing the production of white and red blood cells and mature platelets ».
Are there no alarm bells that can immediately alert doctors?
«The first symptoms can be a general tiredness, a vague malaise which, especially in a young patient, may not worry. In some cases there may be bleeding for example from the nose, petechiae, hematomas. However, when the disease becomes complicated very early with the picture of “disseminated intravascular coagulation”, severe damage to internal organs, such as the brain, can prevent even just starting treatment».
If this disease is diagnosed early, can it be cured?
“If you have a diagnostic suspicion (which must then be confirmed initially with a simple blood test and then the marrow and DNA) A timely administration of retinoic acid, a derivative of vitamin A, may also be sufficient to prevent the fearful hemocoagulative complication of the disease from occurring. But in some cases, their onset is so rapid that it prevents a diagnosis, made even more difficult by its rarity. On average there are only 150 cases a year in Italy».
Michele Merlo, who died at 28: the insights
What are the success rates if the patient is treated immediately? «Mvery high it reaches 93-94%, generally by combining retinoic acid with chemotherapy or arsenic which induces the “death” of cancer cells. It remains, and remains all over the world – indeed Italy is an excellence in the treatment of this pathology – that hard core of 5-6% of patients for whom it is not possible to arrive in time “.
What is the cause of this disease?
“We do not know the original cause – Fabrizio Pane clarifies – but thanks to Italian studies, already in the early nineties, cwe know the mechanism by which it develops, discovered by noting that in some cases acute leukemia responded very well to treatment with retinoic acid, a derivative of vitamin A. Starting from this data, we went to investigate what characterized, at the DNA level, the patients who responded so well to this simple cure and it was seen that they were characterized by the “exchange” between two chromosomes, the number 15 and 17, or rather by the exchange of two of their “pieces” which took place during cell multiplication. This “mistake” causes the production of abnormal proteins that block the production of white blood cells in the bone marrow. In this way, as already mentioned, immature and inefficient white blood cells are created which, however, occupy the “space” that should be destined for mature white blood cells, red ones and platelets. Hence the coagulation problems and bleeding. Retinoic acid prevents abnormal proteins from replicating, thus recovering a situation of normality ».
June 7, 2021 (change June 7, 2021 | 20:15)
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