ABSTRACT
resumen está disponible en el texto completo
Abstract Acute myeloid leukemia (AML) comprises a heterogeneous group of hematopoietic cell neoplasms of myeloid lineage that arise from the clonal expansion of their precursors in the bone marrow, interfering with cell differentiation, leading to a syndrome of bone marrow failure. AML is a consequence of genetic and epigenetic changes (point mutations, gene rearrangements, deletions, amplifications, and arrangements in epigenetic changes that influence gene expression) in hematopoietic precursor cells, which create a clone of abnormal cells that are capable of proliferating but cannot differentiate into mature hematopoietic cells or undergo programmed cell death. The diagnosis requires more than 20% myeloid blasts in the bone marrow and certain cytogenic abnormalities. Treatment will depend on age, comorbidities, and cytogenetic risk among the most frequent.
ABSTRACT
Introducción: El eritema nudoso leproso o leprorreacción de tipo 2 supone una reacción de hipersensibilidad nmunológica humoral de tipo III en la lepra lepromatosa y borderline, la cual se presenta clásicamente como nódulos subcutáneos dolorosos en la piel, además de otras manifestaciones sistémicas. En este reporte de caso se mostrará una forma típica de este tipo de leprorreacción. Presentación del caso: Paciente masculino de 28 anos de edad, con diagnóstico de lepra lepromatosa multibacilar en tratamiento, quien fue remitido al Hospital de San José por presentar cuadro clínico de un mes de evolución de fiebre, malestar general, pérdida de peso y aumento de lesiones nodulares en miembros superiores, con hallazgo adicional de bicitopenia y neutropenia severa. Durante la estancia hospitalaria, es valorado por el Servicio de Dermatología quienes consideran el diagnóstico de eritema nudoso leproso e inician tratamiento; además se evidencia mejoría de la neutropenia con posterior aparición de leucocitosis en aumento y en el frotis de sangre periférica, la presencia de blastos, por lo que se sospecha que el paciente curse con una enfermedad hematolinfoide. Por lo anterior, es valorado por el Servicio de Hematología quienes lo descartan, tratándose entonces de una reacción leucemoide probablemente secundaria a su patología de base. Finalmente, el paciente evoluciona favorablemente y es dado de alta sin complicaciones. Conclusión: La leprorreacción de tipo 2, es una reacción inmune mediada por inmunocomplejos que se presenta típicamente como nódulos subcutáneos dolorosos en la piel, además de presentarse con síntomas constitucionales asociados y daño en otros órganos como hígado, riñón y médula ósea. El conocimiento de las leprorreacciones, el diagnóstico y tratamiento oportunos son fundamentales para prevenir la orbimortalidad en estos pacientes.
Introduction: Erythema nodosum leprosum, or type 2 leprosy reaction, is a type III humoral immunological reaction in the lepromatous and borderline categories of the disease. This classically presents as painful subcutaneous nodules on the skin, and other systemic manifestations. In this case report, a typical form of this type of leprosy reaction will be shown. Case presentation: A 28year-old male patient diagnosed and being treated for multibacillary lepromatous leprosy, was referred to the San Jose Hospital one month after onset. He presented with clinical symptoms such as fever, malaise, weight loss, and increased nodular lesions in the upper limbs, with additional finding of bi-cytopenia and severe neutropenia. He was evaluated by the Dermatology Department during his hospital stay, where he was diagnosed with erythema nodosum leprosum and began treatment for it. A further improvement was observed in the neutropenia, with an increase in leucocytosis. The presence of blasts was found in the peripheral blood smears, which led to the suspicion that the patient had a haemato-lymphoid disease. This was evaluated by the haematology department, but was ruled out. Therefore, this condition corresponded to a leukemoid reaction, probably secondary to the underlying disease. Finally, the patient progressed well and was discharged without complications. Conclusions: Type 2 leprosy reaction is an immune reaction mediated by the immune complex that occurs typically as painful subcutaneous nodules on the skin, in addition to the associated constitutional symptoms and damage to other organs such as liver, kidney and bone marrow. Knowledge of leprosy reactions, and their timely diagnosis and treatment are essential in preventing morbidity and mortality in these patients.
Subject(s)
Humans , Male , Adult , Erythema Nodosum , Leprosy, Lepromatous , Leukemoid ReactionABSTRACT
Estudos epidemiológicos sobre Síndromes Mielodisplásicas (SMD) não são encontrados na literatura brasileira, o que requer investigação dessa doença prevalente em idosos e com incidência maior com o aumento da idade. Esse trabalho objetivou investigar o perfil sociodemográfico e clínico dos pacientes portadores de SMD. Tratase de um corte transversal, desenvolvido no Rio Grande do Norte, realizado de janeiro de 2000 a dezembro de 2010. Para análise descritiva foi utilizado o programa Epi Info 2002, versão 3.5.2. Os cálculos da probabilidade de associação entre as características analisadas e o gênero foram realizados pelos Testes do qui-quadrado, de Fisher e Exato de Fisher. O nível de significância considerado foi de 0,05. O trabalho foi aprovado em seus aspectos éticos e metodológico pelo Comitê de Ética em Pesquisa CEP/HUOL protocolo 432/10. Dos 29 pacientes selecionados, houve predomínio de idosos, do sexo masculino, com baixa escolaridade, que apresentaram anemia como sintoma inicial. A maior parte foi de pessoas de pele branca, residentes em casa própria, moradores em zona urbana e com renda inferior a dois salários mínimos. Todos utilizaram terapia com hemoderivados, principalmente o concentrado de hemácias, numa frequência de quatro ou mais unidades por mês de consumo, sendo que 20% realizou dosagem de ferritina sérica, todos com valores acima do normal referenciado. Conclui-se que se faz necessário a realização de pesquisas com maiores populações, de caráter multicêntrico a fim de melhor evidenciamento dos dados sociodemográficos e clínicos com possibilidade de avaliação por regiões do país.
Epidemiological studies on Myelodysplastic Syndromes (MDS) are not found in Brazilian literature, which requires investigation of this prevalent disease in the elderly and higher incidence with increasing age. This study aimed to investigate the sociodemographic and clinical characteristics of patients with MDS to characterize this population at a referral center for high complexity. It is a cross-performed from January 2000 to December 2010. For descriptive analysis was conducted using Epi Info 2002, version 3.5.2. The calculations of the likelihood of association between the characteristics analyzed and gender were performed using the chi-square, Fisher and Fisher's Exact. The level of significance was 0.05. The study was approved in its ethical aspects and the methodological Ethics Committee in Research ECR/HUOL Protocol 432/10. We selected 29 patients. The sample was characterized mainly by elderly male with lower education, who had anemia as initial symptom. Most were white-skinned people living in their own homes, residents in urban areas and with income less than two minimum wages. All blood products used therapy, especially red blood cells, a frequency of four or more units per month of consumption. Only 20% performed dosage of serum ferritin, all with values referenced above normal. We conclude that it is necessary to conduct research with larger populations, multicenter character in order to best evidence on the demographic data and clinical evaluation with the possibility of the country.
Subject(s)
Preleukemia , Myelodysplastic Syndromes , Anemia, Refractory , Population Dynamics , EpidemiologyABSTRACT
As síndromes mielodisplásicas constituem um grupo de doenças de ordem clonal hematopoética evidenciadas por estudos em todo o mundo. A estimativa de sobrevida dos pacientes e de casos que apresentam evolução leucêmica requer investigação na população brasileira, pois não se conta com nenhum dado dessa natureza. Assim, este estudo objetivou caracterizar e estimar a sobrevida e evolução leucêmica de portadores de síndromes mielodisplásicas acompanhados em um serviço de referência. Trata-se de um estudo de coorte retrospectiva realizada de janeiro de 2000 a dezembro de 2010. Para análise descritiva foi utilizado o programa Epi Info 2002, versão 3.5.2, e para os cálculos das probabilidades de sobrevida foi empregado o método de Kaplan-Meier pelo uso do programa Statistic Package for Social Sciences (SPSS), versão 10.0. Os cálculos da probabilidade de associação entre as características analisadas e o gênero foram realizados pelos testes do qui-quadrado de tendência, de Fisher, Mann Whitney, e de Log Rank. O nível de significância considerado foi de 0,05. O trabalho foi aprovado em seus aspectos ético e metodológico pelo Comitê de Ética em Pesquisa do Hospital Universitário Onofre Lopes (HUOL), sob o Protocolo n. 432/10. Dos 29 pacientes selecionados, houve predomínio de idosos, do sexo masculino, com baixa escolaridade. Apresentaram baixa probabilidade acumulada de sobrevida e índices de evolução leucêmica em torno de 27%, sem nenhum resultado satisfatório para o tratamento quimioterápico, bem como nenhuma indicação de transplante de medula óssea como possibilidade de cura. Fazem-se necessárias pesquisas com populações maiores para caracterização em todo território nacional.
Myelodysplastic syndromes constitute a group of clonal hematopoietic diseases shown by studies all around the world. The survival estimation of the patients and the cases presenting leukemic evolution demand investigation in the Brazilian population, as there's no data with regard to this theme. Thus, this study aimed to characterize and estimate the survival and leukemic evolution of patients with myelodysplastic syndromes followed up in a reference service. This is a retrospective cohort study carried out from January 2000 to December 2010. The software Epi Info 2002, version 3.5.2, was used for descriptive analysis, and for the calculations of survival probabilities the Kaplan-Meier method was employed through the software Statistic Package for Social Sciences (SPSS), version 10.0. The calculations of the association probability between the characteristics analyzed and gender were performed using the chi-square for trend, Fisher, Mann Whitney, and Log Rank tests. The significance level was 0.05. The ethical and methodological aspects of the study were approved by the Research Ethics Committee of HUOL, under the Protocol 432/10. Out of the 29 selected patients, there was a predominance of elderly people, males, with low education. They showed low cumulative probability of survival and leukemic evolution rates around 27%, with no satisfactory outcome from chemotherapy, as well as no indication of bone marrow transplantation as a possible cure. There is a need for researches with larger populations for the characterization all over the national territory.