Midostaurin en combinación de quimioterápia como tratamiento de leucémia mieloblástica aguda con mutación FTL3, a propósito de un caso / Midostaurin in combination with chemotherapy as a treatment for acute myeloblastic leukemia with FTL3 mutation, a case report

La leucemia mieloblástica aguda (LMA) es una enfermedad heterogénea caracterizada por el crecimiento descontrolado de precursores mieloides indiferenciados que provoca un fallo medular. Según datos del programa de Vigilancia, Epidemiología y Resultados Finales (SEER) se estima una incidencia anual de 4,2 por cada 100.000 habitantes. El porcentaje de incidencia en menores de 20 años es 5,1% y en personas entre 65-84 años, es 46,5%. La tasa de supervivencia a 5 años varía en función de la edad de los pacientes, siendo en menores de 20 del 67% y en mayores a dicha edad disminuye al 25%. La leucemia mieloide aguda representa el 40% del total de leucemias y la mediana de edad de los pacientes es 65 años. Las alteraciones citogenéticas más frecuentes son: traslocación (8;21), inversión cromosoma 16, traslocación (16;16), trisomía del cromosoma 8, deleciones en los cromosomas 5 y 7, y mutación en el gen FLT3 (13q12), la cual está presente en el 30% de los nuevos diagnósticos. El tratamiento estándar de quimioterapia sigue basándose en el esquema intensivo 3+7 que consiste en citarabina con antraciclinas. El objetivo de las nuevas terapias en LMA es el tratamiento dirigido debido a los avances en el diagnóstico y la tipificación. (AU)

Acute myeloblastic leukemia (AML) is a heterogeneous disease characterized by uncontrolled growth of undifferentiated myeloid precursors leading to bone marrow failure. According to data from the Surveillance, Epidemiology and End Results (SEER) program, the annual incidence is estimated at 4.2 per 100,000 population. The incidence rate in children under 20 years of age is 5.1% and in people between 65-84 years of age, it is 46.5%. The 5-year survival rate varies according to the age of the patients, being 67% in those under 20 years of age and 25% in those older than 20 years of age. Acute myeloid leukemia represents 40% of all leukemias and the median age of the patients is 65 years. The most frequent cytogenetic alterations are: translocation (8;21), inversion of chromosome 16, translocation (16;16), trisomy of chromosome 8 and deletions in chromosomes 5 and 7, mutation in the FLT3 gene (13q12), which is present in 30% of new diagnoses. Standard chemotherapy treatment is still based on the intensive 3+7 scheme consisting of cytarabine with anthracyclines. The focus of new therapies in AML is targeted therapy due to advances in diagnosis and typing. (AU)

[Metronidazole-induced encephalopathy: description of a case with radiological and anatomopathological findings]. / Encefalopatia inducida por metronidazol: descripcion de un caso con hallazgos radiologicos y anatomopatologicos.

INTRODUCTION: Metronidazole is a widely known and used antibiotic. In exceptional cases, an encephalopathy with characteristic lesions on magnetic resonance imaging (MRI), usually located in the cerebellum and splenium of the corpus callosum, may be an adverse effect. The incidence and pathogenesis are unknown. The suspension of the treatment usually resolves the symptoms and normalizes the MRI in a few weeks. Due to the usual good prognosis, the anatomopathological findings are exceptional. We present a clinical case with the radiological findings suggestive of metronidazole-induced encephalopathy and, exceptionally, we provide the anatomopathological findings. CASE REPORT: A 72 years-old woman with severe Crohn's disease who, months after starting treatment with metronidazole, presented a slowly progressing bradypsychia and difficulty walking until she came to coma. In MRI it showed hyperintense images in T2 in the corpus callosum, red and dentate nuclei. He improved by stopping metronidazole but later developed sepsis and died. At autopsy, softening of the red nucleus was observed and, microscopically, cell necrosis and demyelination. CONCLUSION: With the publication of the clinical, radiological and anatomopathological information of our case we intend to promote the knowledge of this infrequent treatable cause of subacute encephalopathy and provide data that help to clarify its pathogenesis.

TITLE: Encefalopatia inducida por metronidazol: descripcion de un caso con hallazgos radiologicos y anatomopatologicos.Introduccion. El metronidazol es un antibiotico ampliamente conocido y utilizado. En casos excepcionales puede producir como efecto adverso un cuadro de encefalopatia con unas lesiones caracteristicas en la resonancia magnetica, localizadas generalmente en el cerebelo y el esplenio del cuerpo calloso. La incidencia y la patogenia se desconocen. La suspension del tratamiento habitualmente resuelve los sintomas y normaliza la resonancia magnetica en pocas semanas. Debido al habitual buen pronostico, los hallazgos anatomopatologicos son excepcionales. Se presenta un caso clinico con los hallazgos radiologicos sugestivos de la encefalopatia inducida por metronidazol y, de forma excepcional, se aportan los hallazgos anatomopatologicos. Caso clinico. Mujer de 72 años, con enfermedad de Crohn grave, que meses mas tarde de iniciar tratamiento con metronidazol presento de forma lentamente progresiva bradipsiquia y dificultad para caminar hasta llegar al coma. En la resonancia magnetica mostraba caracteristicas imagenes hiperintensas en T2 en el cuerpo calloso, y los nucleos rojos y dentados. Mejoro al suspender el metronidazol, pero posteriormente desarrollo una sepsis y fallecio. En la autopsia se observo reblandecimiento del nucleo rojo y, microscopicamente, necrosis celular y desmielinizacion. Conclusion. Con la publicacion de la informacion clinica, radiologica y anatomopatologica de este caso se pretende fomentar el conocimiento de esta infrecuente causa tratable de encefalopatia subaguda y aportar datos que ayuden a aclarar su patogenia.

Cefalea en jóvenes: características clínicas en una serie de 651 casos / Headache in young patients: Clinical characteristics of a series of 651 cases

Introducción: La cefalea produce un impacto negativo sobre la calidad de vida de los jóvenes. Nuestro objetivo es analizar las características de esta población en una consulta monográfica de cefaleas (CMC) y evaluar la carga de las diferentes cefaleas codificadas según la Clasificación Internacional de Cefaleas (CIC). Métodos: Durante un período de 6 años y medio, se han registrado los pacientes de edades entre los 14 y los 25 años atendidos en la CMC recogiendo de cada uno de ellos el sexo, pruebas complementarias y tratamiento utilizado previamente. Se llevó a cabo la comparación de las características de esta población con la de mayores de 25 años. Resultados: Seiscientos cincuenta y un pacientes de entre 14 y 25 años fueron atendidos durante el período de inclusión; el 95,6% había recibido tratamiento sintomático y el 30,1% tratamiento preventivo. Setecientas cincuenta y cinco cefaleas fueron registradas, 80 fueron cefaleas secundarias, la mayoría codificadas en el grupo 8. El 77,2% de ellas fueron incluidas en el grupo 1, el 3,1% en el grupo 2, el 1,2% en el grupo 3 y el 5% en el grupo 4. El 0,6% de ellas fueron clasificadas en el grupo 13 y el 0,9% en el grupo 14. En 449 pacientes la puntuación del Headache Impact Test (HIT-6) mostró al menos un impacto moderado sobre la calidad de vida. Conclusión: La mayoría de las cefaleas en jóvenes podrían ser codificadas de acuerdo con los criterios de la CIC. La migraña fue el diagnóstico más frecuente. Aunque la cefalea fue comúnmente asociada con impacto negativo en la calidad de vida, los tratamientos preventivos no fueron utilizados extensamente antes de ser derivados a la CMC

Introduction: Headache has a negative impact on health-related quality of life in young patients. We aim to analyse the characteristics of a series of young patients visiting a headache clinic and estimate the burden of different types of headaches listed by the International Classification of Headache Disorders (ICHD). Methods: We prospectively recruited patients aged 14 to 25 years who were treated at our clinic during a period of 6.5 years. We recorded each patient's sex, complementary test results, and previous treatment. We subsequently compared the characteristics of our sample to those of patients older than 25. Results: During the study period, we treated 651 patients aged 14 to 25 years; 95.6% had received symptomatic treatment, and 30.1% had received preventive treatment. A total of 755 headaches were recorded. Only 80 were secondary headaches, most of which were included in Group 8; 77.2% were included in Group 1, 3.1% in Group 2, 1.2% in Group 3, 5% in Group 4, 0.6% in Group 13, and 0.9% in Group 14. According to Headache Impact Test (HIT-6) scores, headache had at least a moderate impact on the quality of life of 449 patients. Conclusion: Most headaches in young patients can be classified according to ICHD criteria. Migraine was the most frequent diagnosis in our sample. Although headache was commonly associated with a negative impact on quality of life, most patients had received little preventive treatment before being referred to our clinic

Headache in young patients: Clinical characteristics of a series of 651 cases. / Cefalea en jóvenes: características clínicas en una serie de 651 casos.

INTRODUCTION: Headache has a negative impact on health-related quality of life in young patients. We aim to analyse the characteristics of a series of young patients visiting a headache clinic and estimate the burden of different types of headaches listed by the International Classification of Headache Disorders (ICHD). METHODS: We prospectively recruited patients aged 14 to 25 years who were treated at our clinic during a period of 6.5 years. We recorded each patient's sex, complementary test results, and previous treatment. We subsequently compared the characteristics of our sample to those of patients older than 25. RESULTS: During the study period, we treated 651 patients aged 14 to 25 years; 95.6% had received symptomatic treatment, and 30.1% had received preventive treatment. A total of 755 headaches were recorded. Only 80 were secondary headaches, most of which were included in Group 8; 77.2% were included in Group 1, 3.1% in Group 2, 1.2% in Group 3, 5% in Group 4, 0.6% in Group 13, and 0.9% in Group 14. According to Headache Impact Test (HIT-6) scores, headache had at least a moderate impact on the quality of life of 449 patients. CONCLUSION: Most headaches in young patients can be classified according to ICHD criteria. Migraine was the most frequent diagnosis in our sample. Although headache was commonly associated with a negative impact on quality of life, most patients had received little preventive treatment before being referred to our clinic.

[Nasal congestion as an autonomic symptom accompanying migraine attacks]. / Congestion nasal como sintoma autonomico acompañante de crisis migranosas.

TITLE: Congestion nasal como sintoma autonomico acompañante de crisis migranosas.

Real-life data in 115 chronic migraine patients treated with Onabotulinumtoxin A during more than one year.

BACKGROUND: OnabotulinumtoxinA (OnabotA) is effective in Chronic Migraine (CM) during first year of treatment and longer. In real clinical setting, CM patients with acute Medication Overuse (MO) or concurrently receiving oral preventatives are treated with OnabotA. We aim to assess evolution of CM patients beyond first year on OnabotA. METHODS: Data were retrospectively collected in three headache units. We analyzed cases who had received at least five sessions of OnabotA according to PREEMPT protocol. We continued OnabotA therapy when a reduction of number of headache days of at least 30% was achieved. RESULTS: We included 115 patients (98 females, 17 males) who completed 7.6 ± 2.3 (5-13) OnabotA procedures. Previously they had not responded to topiramate and, at least, one other preventative. Age at inclusion was 45.3 ± 12 (14-74) years, and latency between CM onset and OnabotA therapy was 43.1 ± 38.2 (6-166) months. At first OnabotA session 92 patients (80%) fulfilled MO criteria and 107 (93%) received a concurrent oral preventative. In 42 cases (36.5%) OnabotA dose was increased over 155 units. After first year in 57 out of 92 patients (61.9%) MO was discontinued. Among those receiving preventatives, in 52 out of 107 they were retired (48.6%). In 22 cases (19.1%) OnabotA administration was delayed to the fourth or fifth month and in 12 (10.4%) it was temporally stopped. Finally, in 18 patients (15.7%) OnabotA was discontinued due to lack of efficacy beyond first year of treatment. CONCLUSION: Our results suggest that discontinuation of acute medication overuse and oral preventive therapies are achievable objectives in long-term using of OnabotA in CM patients.

[The effectiveness of rituximab in refractory autoimmune thrombocytopenic purpura and haemolytic anaemia]. / Efectividad de rituximab en púrpura tombocitopénica y anemia hemolítica autoinmune refractarias.

OBJECTIVE: To evaluate the efficacy and safety of treatment with rituximab in patients presenting autoimmune thrombocytopenic purpura and haemolytic anaemia. METHOD: A check was carried out of the medical records of the patients starting treatment with rituximab for compassionate use in 2004 at doses of 375 mg/m2 per week for 4 weeks. The rate of patients achieving full response in accordance with the best criteria found in the bibliography was assessed. All adverse reactions described in the medical records were gathered. RESULTS: Six patients with thrombocytopenic purpura were candidates for treatment. Five began treatment, four of them completed treatment, and three of these patients achieved full response. This response was achieved at different times and was sustained for at least six months. Two patients with autoimmune haemolytic anaemia were treated and both achieved full response again at different times and in this case, it was sustained for at least 8 months. One patient suffered mild adverse reactions to treatment. CONCLUSIONS: Rituximab is a new perspective for the treatment of refractory autoimmune cytopenias, and has a good safety profile.

Efectividad de rituximab en púrpura tombocitopénica y anemia hemolítica autoinmune refractarias / No disponible

Objetivo: Evaluar la efectividad y seguridad del tratamiento con rituximab en pacientes con púrpura trombocitopénica y anemia hemolítica autoinmune. Método: Se han revisado las historias clínicas de los pacientes que iniciaron tratamiento con rituximab como uso compasivo en el año 2004 a dosis de 375 mg/m2 semanal durante 4 semanas. Se evaluó la tasa de pacientes que alcanzó respuesta completa según los mejores criterios encontrados en la bibliografía. Se recogieron las reacciones adversas descritas en la historia clínica. Resultados: Seis pacientes con púrpura trombocitopénica autoinmune fueron candidatos a tratamiento. Cinco iniciaron tratamiento, cuatro de ellos completaron el tratamiento, de los cuales tres obtuvieron respuesta completa. Dicha respuesta es alcanzada en diferentes tiempos y es mantenida al menos durante 6 meses. Dos pacientes con anemia hemolítica autoinmune fueron tratados y ambos alcanzaron respuesta completa también en diferentes tiempos y esta se mantuvo al menos durante 8 meses. Las reacciones adversas al tratamiento que sufrió algún paciente fueron leves. Conclusiones: Rituximab es una nueva expectativa al tratamiento de citopenias autoinmunes refractarias, con un buen perfil de seguridad

Objective: To evaluate the efficacy and safety of treatment with rituximab in patients presenting autoimmune thrombocytopenic purpura and haemolytic anaemia. Method: A check was carried out of the medical records of the patients starting treatment with rituximab for compassionate use in 2004 at doses of 375 mg/m2 per week for 4 weeks. The rate of patients achieving full response in accordance with the best criteria found in the bibliography was assessed. All adverse reactions described in the medical records were gathered. Results: Six patients with thrombocytopenic purpura were candidates for treatment. Five began treatment, four of them completed treatment, and three of these patients achieved full response. This response was achieved at different times and was sustained for at least six months. Two patients with autoimmune haemolytic anaemia were treated and both achieved full response again at different times and in this case, it was sustained for at least 8 months. One patient suffered mild adverse reactions to treatment. Conclusions: Rituximab is a new perspective for the treatment of refractory autoimmune cytopenias, and has a good safety profile

Virgin olive oil and coenzyme Q10 protect heart mitochondria from peroxidative damage during aging.

The mitochondrial theory of aging suggests that this phenomenon is the consequence of random somatic mutations in mitochondrial DNA, induced by long-term exposure to free radical attack. There are two potential dietary means of delaying the effects of free radicals on cellular aging, i.e., enrichment of mitochondrial membranes with monounsaturated fatty acids and supplementation with antioxidants. We have performed a preliminary study on male rats, 6 or 12 month old, fed with diets differing in the nature of the fat (virgin olive oil or sunflower oil) and/or with antioxidant supplementation (coenzyme Q10), analysing hydroperoxide and coenzyme Q9 and Q10 in heart mitochondria. Preliminary results allow us to conclude that the CoQ10 dietetic supplementation as well as the enrichment of the cellular membranes with monounsaturated fatty acids, successfully protect mitochondrial membranes from aged rats against the free radical insult.