RESUMO
Improvements in clinical assessment have occurred since the last published recommendations on the diagnosis and treatment of acute promyelocytic leukemia in 2013. Here, a committee of specialists of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy presents a comprehensive review on the current knowledge, focusing on the advances in diagnosis, risk assessment, and frontline and salvage therapy. The concept of urgent diagnosis is explored as well as the management of critical situations such as coagulopathy and differentiation syndrome. Recent adjustments in risk stratification based on white blood cell counts only are presented together with the incorporation of chemo-free regimens for non-high-risk patients. Special conditions such as acute promyelocytic leukemia in children, the elderly and pregnant women are discussed. Finally, acute promyelocytic leukemia is presented as a highly curable disease because of the real possibility of targeted therapy towards differentiation, and, paradoxically, as a serious and urgent condition that deserves prompt recognition and management to avoid early mortality.
RESUMO
A Leucemia Mielóide Crônica (LMC), cuja incidência é de um a dois casos para cada 100 mil habitantes por ano, corresponde de 15 por cento a 20 por cento das leucemias. É uma doença mieloproliferativa crônica clonal, caracterizada por leucocitose com desvio à esquerda, esplenomegalia e pela presença do cromossomo Philadelphia (Ph), que resulta da translocação recíproca e equilibrada entre os braços longos dos cromossomos 9q34 e 22q11, gerando a proteína híbrida BCR-ABL, com atividade aumentada de tirosino quinase. A proteína BCR-ABL está presente em todos os pacientes com LMC, e sua hiperatividade desencadeia liberação de efetores da proliferação celular e inibidores da apoptose, sendo sua atividade responsável pela oncogênese inicial da LMC. A doença evolui em três fases: crônica, acelerada e aguda. Na fase crônica (FC) ocorre proliferação clonal maciça das células granulocíticas, mantendo estas a capacidade de diferenciação. Posteriormente, num período de tempo variável, o clone leucêmico perde a capacidade de diferenciação e a doença passa a ser de difícil controle (fase acelerada - FA) e progride para uma leucemia aguda (crise blástica - CB). Nesse artigo discutimos a história natural e as definições das fases da doença, de acordo com os critérios mais utilizados.
Chronic myeloid leukemia (CML) is estimated at approximately 1 to 2 cases per 100,000 individuals and accounts for approximately 15 percent to 20 percent of all patients with leukemia. CML is a clonal disease characterized by balanced translocation between chromosomes 9 and 22 (Philadelphia chromosome). The resulting BCR-ABL gene has abnormal tyrosine kinase activity which stimulates cell growth and is responsible for the transformed phenotype of CML cells. The disease is characterized by a triphasic course that includes a chronic phase (CP), an accelerated phase (AP) and an acute or blastic phase (BP). Unless the disease is controlled or eliminated, patients progress to AP and BF in variable periods of time. Several staging classification systems are used for CML all of which were designed in the pre-imatinib era. In this article we discuss the natural history of CML and phase definitions according to the most useful criteria.
Assuntos
Humanos , Leucemia Mielogênica Crônica BCR-ABL Positiva , Leucemia Mielogênica Crônica BCR-ABL Positiva/classificação , Leucemia Mielogênica Crônica BCR-ABL Positiva/históriaRESUMO
We report an increased incidence of high relapse risk features in 157 APL Brazilian patients. Out of 134 patients treated with ATRA and anthracyclines, only 91 (67.9%) achieved remission because 43 (32%) died during induction. The death rate during consolidation was 10.5%. Bleeding complications were the most frequent cause of failure (21.6%).
