It accounts for 10-15% of all cases of acute myeloid leukemia and is considered one of the most aggressive blood cancers due to the serious clinical manifestations it induces, among which life-threatening haemorrhages stand out.
The average age of onset of acute promyelocytic leukemia is around 30-40 years and can affect individuals of both sexes equally.
For further information: Leukemia: general approach to the disease and environmental factors).Similarly to other forms of acute myeloid leukemia, fulminant leukemia is also attributable to acquired genetic alterations. More specifically, people who are affected by acute promyelocytic leukemia have an acquired translocation between chromosomes 15 and 17 (in other words, there is a sort of exchange of genetic material between the two chromosomes). This chromosomal translocation involves the formation of an abnormal fusion protein (PML / RAR-alpha) and, more specifically, of an abnormal retinoic acid receptor capable of causing a block in the maturation of myeloid stem cells which therefore remain at the promyelocyte stage (hence the name of the disease itself).
For further information: Leukemia - Causes and Symptoms , fatigue, malaise, paleness and bleeding. It is precisely hemorrhages that represent the greatest danger in the case of fulminant leukemia. In fact, the onset of the disease can be characterized by a sudden onset associated with severe bleeding symptoms due to a reduced number of platelets and to the alteration of coagulation mechanisms. In this regard, it is estimated that about 10-20% of patients who develop the disease undergo fatal major bleeding - such as, for example, brain bleeding - even before receiving the correct diagnosis and undergoing treatment.
However, the hemorrhages that arise are not always localized in the brain; in fact, skin hemorrhages, nosebleeds, bleeding from the gums or haemorrhages in the gastrointestinal or genitourinary tract can also occur.
Fatigue and a general feeling of malaise are symptoms often present in patients who undergo the development of fulminant leukemia.
For further information: Leukemia Symptoms and physical examination of the patient to evaluate the symptoms and clinical manifestations presented, as well as the general state of health.
Subsequently, the patient will have to undergo careful analysis to confirm or not the presence of the disease that the doctor suspects. More specifically, it will be necessary to perform a complete blood count and peripheral blood smear for morphological evaluation.
To confirm the diagnosis, analyzes are carried out to search for the characteristic cytogenetic and molecular anomalies, a distinctive feature of fulminant leukemia.
Timely diagnosis is essential to be able to intervene in time and be able to treat this pathology, thus greatly increasing the patient's hopes of survival.
To learn more about the methods of diagnosis: Leukemia: the diagnosis of cancer does not play the same role they play in the treatment of other types of cancer. The current therapeutic approach, in fact, involves the administration of retinoic acid (a derivative of vitamin A) and arsenic trioxide which act in a complementary way: retinoic acid helps to complete the differentiation of promyelocytes into mature neutrophils (white blood cells more abundant in the blood), while arsenic trioxide induces apoptosis (programmed cell death) of cancer cells.
In addition to the above approach, fulminant leukemia can also be treated with transfusions of platelet concentrates, fresh frozen plasma and blood products. Furthermore, it will be up to the doctor to evaluate, case by case, the need or not to resort to the use of any chemotherapy drugs.
Of course, all necessary supportive therapies will also have to be adopted.
