Follow-up and outcome of the twelve-year experience in adult patients with acute promyelocytic leukemia

ABSTRACT Acute promyelocytic leukemia is a subtype of acute myeloid leukemia, characterized by the presence of neoplastic promyelocytes, due to the reciprocal balanced translocation between chromosomes 15 and 17. Currently, with the use of agents that act directly on this molecular change, such as all-trans retinoic acid and arsenic trioxide, APL has shifted from a highly mortal to a curable disease. However, some cases are still at high risk of death, especially early death, and acquiring a better understanding of the clinical and biological factors involving APL is needed to correctly identify and treat such cases. The early suspected diagnosis and prompt initiation of the target therapy are important for better response rates. The follow-up and outcomes, using real-life data from 44 consecutive APL patients, were studied between 2001 and 2013. The overall survival rate was 82.7% and early death was 16%. Almost all patient deaths were due to severe bleeding, which was confirmed by multivariate analysis, as the most important prognostic factor leading to death. A better understanding the pathogenesis of the hemorrhagic complications in APL is needed, as well as the risk factors associated with early death in APL patients, as this has become synonymous with overall mortality.

Profile of blood donations with a positive serology in Southern Brazil

ABSTRACT Objective: The aim of this study was to describe the characteristics of blood donors and the serological profile of the blood donations at the blood bank of the University Hospital Polydoro Ernani de São Thiago of the Federal University of Santa Catarina from January 2011 to December 2016. Methods: The characteristics of donors and the serological results of the donated blood were compiled from databases. Only donations with a negative serology or a positive serology confirmed by second-sample testing were included in the study. Results: A total of 14,368 donations were included in the study, of which 118 (0.8%) had a confirmed positive serology. Of the total donations, 94.3% were from spontaneous donations and 5.7% from replacement donation. Donations were predominantly from men (54.1%), individuals aged 18 to 29 years (69.1%), and repeat donors (47.7%). Detection rates were higher for HBV (0.63%), followed by syphilis (0.13%), HIV (0.05%), HCV (0.02%), and Chagas disease (0.01%). With the exception of HIV, positive results were more frequent in the older age groups. Positive results for HBV, HCV, and HIV were more frequent among first-time donors. Replacement donations were more likely to have HBV (OR 7.7; 95% CI 4.9-12.1, p < 0.0001) and HIV (OR 6.7; 95% CI 1.3-34.7; p = 0.02) than spontaneous donations. Conclusion: This study showed that the frequency of infections in blood donations at the HU-UFSC blood bank was lower than the national estimates and that our population may have a greater prevalence of syphilis among older donors

Analysis of the presence of FLT3 gene mutation and association with prognostic factors in adult and pediatric acute leukemia patients

ABSTRACT When the FLT3 gene is mutated, it originates a modified receptor with structural changes, which give survival advantage and malignant hematopoietic cell proliferation. Thus, the presence of mutations in this gene is considered an unfavorable prognostic factor. A total of 85 consecutive samples of newly diagnosed untreated patients with AL were included in the study after they provided their informed consent. FLT3 gene mutations were detected by PCR. For the pediatric group, a positive correlation was observed between WBC count and the presence of FLT3-ITD in patients with AML and ALL. Furthermore, children with AML who had the FLT3-ITD mutation showed a tendency to express CD34 in blast cells. In the adult group, the AML patients with FLT3-ITD who expressed CD34 in blast cells had a tendency to worse progression. The present data indicate no association between the prognostic factors evaluated and FLT3 gene mutations in adult with AL. Yet, the presence of FLT3-ITD mutation was significantly related with WBC count in the pediatric group. These findings demonstrate that FLT3 gene mutations can be considered as independent poor prognostic factors.

Mature B cell neoplasms: retrospective analysis of 93 cases diagnosed between 2011 and 2014 in a University hospital in southern Brazil

BACKGROUND: According to the 2008 World Health Organization classification, mature B-cell neoplasms are a heterogeneous group of diseases that include B-cell lymphomas and plasma cell disorders. These neoplasms can have very different clinical behaviors, from highly aggressive to indolent, and therefore require diverse treatment strategies. OBJECTIVE: The aim of this study was to assess the profile of 93 patients diagnosed with mature B-cell neoplasms monitored between 2011 and 2014. METHODS: A review of patients' charts was performed and laboratory results were obtained using the online system of the Universidade Federal de Santa Catarina. RESULTS: The study included 93 adult patients with mature B-cell neoplasms. The most frequent subtypes were multiple myeloma, chronic lymphocytic leukemia, diffuse large B-cell lymphoma, follicular lymphoma, and Burkitt's lymphoma. The median age at diagnosis was 58 years with a male-to-female ratio of 1.3:1. There were statistical differences in terms of age at diagnosis, lactate dehydrogenase activity and Ki-67 expression among the subtypes of B-cell lymphoma. According to the prognostic indexes, the majority of multiple myeloma patients were categorized as high risk, while the majority of chronic lymphocytic leukemia patients were classified as low risk. CONCLUSIONS: This study demonstrates the profile of patients diagnosed with mature B-cell neoplasms in a south Brazilian university hospital. Of the B-cell lymphoma, Burkitt's lymphoma presented particular features regarding lactate dehydrogenase activity levels, Ki-67 expression, age at diagnosis, and human immunodeficiency virus infection.

Frequencies of polymorphisms of the Rh, Kell, Kidd, Duffy and Diego systems of Santa Catarina, Southern Brazil

Background Red blood cell genes are highly polymorphic with the distribution of alleles varying between different populations and ethnic groups. The objective of this study was to investigate gene polymorphisms of blood groups in the state of Santa Catarina, Southern Brazil. Methods Three hundred and seventy-three unrelated blood donors and 31 transfusion-dependent patients were evaluated to investigate polymorphisms of the Rh, Kell, Duffy, Kidd, and Diego blood group systems in a population from the state of Santa Catarina. The subjects, from seven regions that comprise the blood-banking network of the state, were assessed between August 2011 and March 2014. The genotypes of the Rh, Kell, Duffy, Kidd, and Diego systems were determined using the restriction fragment length polymorphism-polymerase chain reaction and allele-specific polymerase chain reaction techniques. Results The genotype frequencies in this study were significantly different when populations from different regions of Santa Catarina were compared. Furthermore, there were also significant differences in the genetic frequencies compared to other Brazilian states. The genotype frequencies of the Kell and Kidd blood groups are similar to European populations from Naples, Italy and Zurich, Switzerland. Conclusion This article reports for the first time the frequency of polymorphisms of blood group systems in blood donors from Santa Catarina, Southern Brazil.

Antiplatelet activity of Croton celditifolius / Atividade antiplaquetária do Croton celtidifolius

Croton celtidifolius Baill is a tree found in the Atlantic Forest South of Brazil, mainly in Santa Catarina. The bark and leaf infusions of this medicinal plant have been popularly used for the treatment of inflammatory diseases. The anti-aggregant activity of C. celtidifolius crude extract (CE) and the column chromatography (CC) isolated compounds flavonoids, catechin and gallocatechin were evaluated in human blood platelets. The platelet-rich plasma (PRP) was incubated with different concentrations of flavonóides (50 - 200 µg/mL) to be tested before platelet aggregation was induced by the agonists adenosine 5'diphosphate (ADP) and collagen. At 200 µg/mL the CE, catechin and gallocatechin markedly inhibited platelet aggregation with the aggregant agents. Using ATP production as an index of platelet secretory capacity, we observed a decreased production of ATP in platelets treated with flavonoids when stimulated by collagen. On the other hand, the flavonoids did not promote inhibitory effect on prothrombin time (PT), thromboplastin time (APTT) and thrombin time (TT). In conclusion, these observations suggest that C. celtidifolius is likely to exert an inhibitory action on platelets in vitro by suppressing secretion and platelet aggregation.

Croton celtidifolius Baill é uma árvore encontrada na Mata Atlântica, no sul do Brasil, principalmente no estado de Santa Catarina. A infusão da casca e folhas dessa planta medicinal é utilizada na medicina popular para o tratamento de doenças inflamatórias. A atividade antiagregante do extrato bruto de C. celtidifolius (CE) e de seus flavonóides isolados por coluna cromatográfica (CC), catequina e galocatequina, foi avaliada em plaquetas humanas. O plasma rico em plaquetas (PRP) foi incubado com diferentes concentrações dos flavonóides testados (50 - 200 µg/mL) e posteriormente a agregação foi induzida pelos agonistas adenosina 5'difosfato (ADP) e colágeno. Na concentração de 200 µg/mL o CE, a catequina e a galocatequina inibiram a agregação plaquetária induzida pelos agonistas. A produção de ATP foi utilizada como um índice de capacidade de secreção plaquetária e observamos uma diminuição na produção de ATP nas plaquetas tratadas com os flavonóides e estimuladas com o colágeno. Por outro lado, os flavonóides não promoveram um efeito inibitório no tempo de protrombina (PT), tempo de tromboplastina parcial ativada (APTT) e tempo de trombina (TT). Essas observações sugerem que o C. celtidifolius exerce, in vitro, uma ação inibitória nas plaquetas através da inibição da secreção e agregação plaquetária.