Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso / Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report

El priapismo es una erección dolorosa y persistente acompañada o no de estímulo sexual. Una causa poco frecuente de esta anormalidad es la leucemia mieloide crónica. Se han reportado pocos casos de priapismo como manifestación inicial de una leucemia de este tipo en pacientes adolescentes. A continuación, se informa el caso de un paciente de 16 años de edad que presentó priapismo como manifestación inicial de una leucemia mieloide crónica. Durante su evolución, no se realizó aspiración de los cuerpos cavernosos. Se inició tratamiento hematológico específico y, ante la persistencia del priapismo, fue necesario realizar un shunt de cuerpos cavernosos en dos ocasiones, tratamiento a pesar del cual existen altas probabilidades de secuelas.

Priapism is a painful and persistent erection, with or without sexual stimulation. A rare cause of such abnormality is chronic myeloid leukemia. Few cases of priapism as an initial manifestation of this type of leukemia have been reported in adolescent patients. Here we describe the case of a 16-year-old patient who presented with priapism as the initial manifestation of chronic myeloid leukemia. No cavernosal aspiration was performed. A specific hematological treatment was started and, given the persistence of priapism, the patient required 2 corpora cavernosa shunt procedures; despite this treatment, there is a high probability of sequelae.

Cefalea atribuida a ictus isquémico. Actualización semiológica y diagnóstica / Headache attributed to ischaemic stroke. An update on its semiology and diagnosis

Introducción: La cefalea es un síntoma frecuente tras el ictus isquémico agudo. Su identificación y diagnóstico constituyen un reto por el perfil de paciente y los criterios diagnósticos actuales de esta entidad. Los objetivos del estudio fueron determinar la prevalencia de cefalea atribuida a ictus isquémico y su forma persistente, y analizar las variables clinicodemográficas y el grado de cumplimiento de los criterios de la Clasificación Internacional de Cefaleas (ICHD-III). Pacientes y métodos: Es un estudio observacional analítico de cohortes prospectivo de pacientes ingresados con ictus isquémico agudo en la unidad de ictus de un hospital de tercer nivel en un período de 12 meses. Resultados: Se incluyó a 244 pacientes con ictus isquémico agudo (el 59,8%, varones; edad media: 71 ± 12,8 años). El 23,2% presentó cefalea en el momento del ingreso o bien en las primeras 72 horas y el 12,5% de ellos presentó cefalea persistente atribuida a ictus isquémico. El 62,5% cumplió los criterios diagnósticos de acuerdo con la ICHD-III. Conclusión: La cefalea tras el ictus isquémico es un síntoma frecuente. Su aparición se asoció al sexo femenino, al ictus de territorio vertebrobasilar y a puntuaciones bajas en la National Institutes of Health Stroke Scale. Sería recomendable revisar los criterios diagnósticos actuales.(AU)

Introduction: Headache is a common symptom in acute ischemic stroke which is often overlooked and undertreated because of focus in neurologic function, communication difficulties in stroke patients and the current diagnostic criteria of this type of headache. The present study aimed to determine the prevalence of Acute and Persistent Headache Attributed to Ischemic Stroke and to analyze the fulfillment of the criteria of the International Classification of Headaches (ICHD-IID). Patients and methods: Prospective observational analytical cohort study. The study population consisted of patients with acute ischemic stroke admitted to the Stroke Unit of a tertiary care hospital over a period of 12 months. Results: Two hundred and forty-four patients with acute ischemic stroke (59.8% males, mean age 71+12.8 years) were included. Headache at onset or at the first 72 hours was present in 23.2% and 12.5% of them presented persistent headache attributed to ischemic stroke. Only 62.5% of the headaches at stroke onset fulfilled the diagnostic criteria of ICHD-III. Conclusion: Headache after ischemic stroke is a common symptom. It was associated with female sex, posterior circulation stroke and low scores on the National Institutes of Health Stroke Scale (NIHSS). The current diagnostic criteria should be reviewed.

Autoestima en personas con daño cerebral adquirido según práctica de actividad física / Self-esteem in people with acquired brain injury according to physical activity

La literatura científica señala la importancia de la actividad física (AF) en las personas con daño cerebral adquirido (DCA) para mantener su salud y funcionalidad; sin embargo, en general presentan secuelas crónicas, bajos niveles de AF y baja autoestima. Con el objetivo de analizar la autoestima en personas con DCA en fase crónica en función de la práctica de AF, se realizó un estudio quasi-experimental ex post facto, comparando dos grupos: personas que realizaban AF(n=50)y personas que no realizaba AF(n=49), evaluando la autoestima con la Escala de Rosenberg. Se realizó un análisis descriptivo y se utilizó la prueba T para muestras independientes. Así, el grupo que realizó AF mostró mejor autoestima y mayor autonomía personal (p≤0.05). Por ello, se recomienda la práctica de AF para mejorarla autoestima en personas con DCA. Además, haber practicado AF antes del DCA parece ser determinante para su práctica a posteriori. (AU)

People with acquired brain injury (ABI) have long-term consequences and show low levels of physical activity (PA), presenting worse self-esteem. Nevertheless, it is proven that the practice of PA in people with ABI improve psychological dimension. The aim was to study if self-esteem in people with ABI in the chronic phase is influenced by PA practice. A quasi-experimental ex post facto design was used, where there were two groups: PA group (n=50) and the sedentary group (n=49). The Rosenberg Scale was used to measure self-esteem. A descriptive analysis was carried out and the T test for independent samples was used. People who practice PA perceived better self-esteem and higher autonomy (p ≤0.05). Therefore, the practice of PA is recommended to improve self-esteem in people with ABI. Furthermore, having practiced PA before the ABI seems to be decisive for its subsequent practice. (AU)

El daño cerebral en los estadios crónicos y la importancia de la calidad de vida como eje de trabajo / Brain damage in chronic stages and the importance of quality of life as an axis of work

El Daño Cerebral Adquirido (DCA) supone una situación de gran trascendencia personal, familiar y social. Sus secuelas implican dificultades en la integración normalizada del individuo, con respecto a su vida previa al daño y dificulta su participación en la sociedad, donde ha cambiado su rol. Las estrategias de atención al DCA varían atendiendo al momento y ámbito en que se sitúan. Durante la fase aguda, la subaguda y la atención crónica, las estrategias están muy ligadas a su etiología, pero una vez superadas estas fases, otras variables como el contexto psicosocial y laboral del afectado/a y su familia pasan a tomar relevancia. Se deben revisar las situaciones personales y familiares para atender a su grado de dependencia. Este análisis debe incluir instrumentos de evaluación que, desde una perspectiva integral, engloben todas las áreas de la vida que son importantes para las personas, y analice la calidad de vida de éstas. Es fundamental en este momento, construir conjuntamente con las personas con DCA, sus familias y los profesionales sociosanitarios, un nuevo proyecto de vida centrado en el bienestar emocional, físico y social, desde las diversas alternativas de recursos para los estadios crónicos con los que se cuenta actualmente en España (AU)

A stroke is a situation of great personal, family, and social significance. The sequel cause problems to integrate the individual at his life before the stroke, and this is difficult to participate in society, where his role has changed. The strategies, in case of accident, are differents depending on time and ambit. The etiology is very important in the acute phase, post acuse phase and chronic phase, however, after chronic phase the etiology less important and the variables such as the psychosocial and work context of the affected person and his family are more important. Personal and family situations should be reviewed to pay attention to the degree of dependency. The analysis must include evaluation instruments that, from a integrative perspective, includes all areas of life important to people, and analyze their quality of life. At this time, it is essential to build a life proyect for people with stroke and their families, focused on emotional, physical and social aspects, using the resource and alternatives that are in Spain to people with a chronic stroke

Patients' perceptions about diagnosis and treatment of chronic myeloid leukemia: a cross-sectional study among Brazilian patients / Percepções dos pacientes sobre diagnóstico e tratamento da leucemia mieloide crônica: estudo transversal entre pacientes brasileiros

CONTEXT AND OBJECTIVES: Chronic myeloid leukemia (CML) requires strict daily compliance with oral medication and regular blood and bone marrow control tests. The objective was to evaluate CML patients' perceptions about the disease, their access to information regarding the diagnosis, monitoring and treatment, adverse effects and associations of these variables with patients' demographics, region and healthcare access. DESIGN AND SETTING: Prospective cross-sectional study among CML patients registered with the Brazilian Lymphoma and Leukemia Association (ABRALE). METHODS: CML patients receiving treatment through the public healthcare system were interviewed by telephone. RESULTS: Among 1,102 patients interviewed, the symptoms most frequently leading them to seek medical care were weakness or fatigue. One third were diagnosed by means of routine tests. The time that elapsed between first symptoms and seeking medical care was 42.28 ± 154.21 days. Most patients had been tested at least once for Philadelphia chromosome, but 43.2% did not know the results. 64.8% had had polymerase chain reaction testing for the BCR/ABL gene every three months. 47% believed that CML could be controlled, but 33.1% believed that there was no treatment. About 24% reported occasionally stopping their medication. Imatinib was associated with nausea, cramps and muscle pain. Self-reported treatment adherence was significantly associated with normalized blood count, and positively associated with imatinib. CONCLUSIONS: There is a lack of information or understanding about disease monitoring tools among Brazilian CML patients; they are diagnosed quickly and have good access to treatment. Correct comprehension of CML control tools is impaired in Brazilian patients.

CONTEXTO E OBJETIVOS: Leucemia mieloide crônica (CML) exige estrita adesão à medicação oral e ao monitoramento do sangue e da medula. O objetivo foi avaliar percepções de pacientes com leucemia mieloide crônica (LMC) sobre a doença, seu acesso à informação sobre diagnóstico, monitoramento e tratamento, efeitos adversos e a associação destes com dados demográficos, geográficos e de acesso a tratamento. DESENHO E LOCAL: Estudo prospectivo transversal realizado com pacientes de LMC cadastrados na Associação Brasileira de Leucemia e Linfoma (Abrale). MÉTODOS: Pacientes com LMC recebendo tratamento do sistema público de saúde foram entrevistados por telefone. RESULTADOS: Entre os 1.102 pacientes entrevistados, os sintomas mais frequentemente levando à busca de consulta foram fraqueza e fadiga. Um terço foi diagnosticado por exames de rotina. O tempo entre sintoma inicial e procura por ajuda foi de 42,28 ± 154,21 dias. A maioria foi testada pelo menos uma vez para o cromossomo Filadélfia, mas 43,2% não sabiam os resultados. 64,8% fizeram exame de reação em cadeia da polimerase para o gene BCR/ABL a cada três meses. 47% acreditavam que LMC pode ser controlada, mas 33,1% acham que não há tratamento. Cerca de 24% disseram que ocasionalmente interrompem o tratamento. Imatinibe associou-se com náusea, câimbra e dor muscular. Aderência auto-reportada associou-se significativamente com hemograma normal e positivamente com uso de imatinibe. CONCLUSÕES: Falta informação ou compreensão sobre monitoramento entre pacientes com LMC; eles recebem diagnóstico rapidamente e têm bom acesso ao tratamento. A correta compreensão das ferramentas de controle em LMC está prejudicada entre eles.

Uso de recursos sanitarios para tratar la leucemia mieloide crónica en Perú / Use of healthcare resources for treating chronic myeloid leukemia in Peru

En el presente artículo original se describen y comentan los datos aportados por un grupo de oncohematólogos, acerca del uso actual de los recursos sanitarios para el tratamiento de la leucemia mieloide crónica y de los eventos adversos relacionados con ésta, en Perú. Mediante la técnica de consenso Mini Delphi, los panelistas lograron elevado grado de acuerdo sobre los temas de interés. A modo de síntesis, se observó distribución de los recursos generales disponibles acorde con las necesidades de cada fase de la leucemia y en adhesión a las pautas terapéuticas internacionales, aunque se detectaron algunas instancias que podrían optimizarse. Resultó evidente la notable disminución de la realización de trasplantes de médula ósea, en Perú. Esta información puede constituir un punto de partida para futuros estudios adicionales en el contexto de la práctica clínica en Latinoamérica. Además, aplicada a modelos farmacoeconómicos apropiados, los datos podrían agilizar la toma de decisiones acorde a la realidad local, por parte de los distintos responsables de los sistemas de salud, para permitir a los enfermos con LMC acceder a las opciones terapéuticas más ventajosas y, aún más trascendente, mejorar su calidad de vida y supervivencia.

This original article details and discusses the information provided by a group of hematologists, about the current use of health resourcesin the management of chronic myeloid leukemia and its treatment related adverse events in Peru. By applying the Mini Delphi consensus technique, a high degree of agreement about the issues of interest was reached among the faculty. To summarize, although it was noticed that the allocation of health resources matches the requirement for each phase in the management of leukemia and it adheres to international guidelines, some topics that could be optimized were identified. A remarkable reduction of bone marrow transplant procedures was evident. These data may represent a starting point for further studies in the clinical practice setting in Latin America. Moreover, using appropriate pharmacoeconomic models, the information obtained may speed up the decision making process according to the local circumstances by those responsible for sanitary assistance, allowing patients with CML to reach the most convenient therapeutic options and, more importantly, improving their quality of life and survival.

Leucemia Mielóide Crônica: história natural e classificação / Chronic Myeloide Leukemia: natural history and classification

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.

Efeito protetor do nifurtimox contra a reativação parasitária em pacientes cronicamente infectados pelo Trypanosoma cruzi e tratados com corticoide em virtude de afecçoes associadas / Nifurtimox as a prophylactic drug to prevent reactivation in chronic chagasic patients treated with corticoid for associated diseases

Pacientes na fase crônica da doença de Chagas foram tratados com corticóide em virtude de afecções associadas e, a fim de tentar coibir reativação da infecção pelo Trypanosoma cruzi, houve uso concomitante do nifurtimox. Levando em conta o verificado em pesquisa anterior, quando corticóide de fato promoveu aumento da parasitemia detectada pelo xenodiagnóstico, pôde ser notado que o nifurtimox mostrou-se apto a evitar a citada acentuação parasitária, podendo tal constatação ser útil em procedimentos assistenciais, quando circunstancialmente estiverem presentes doença de Chagas e imunodepressão.

Patients in the chronic phase of Chagas' disease and receiving corticoid because of concommitant diseases were treated with nifurtimox. We proved in another paper that in the chronic phase of Chagas' disease corticoid use is associated with increased parasitemia, as evaluated by xenodiagnosis. In this study nifurtimox use prevented this increase, and we suggest that in immunocompromised patients with chronic Chagas' disease the use of this drug could be useful.