Secondary hypogammaglobulinemia in adults—A large retrospective cohort study / Hipogammaglobulinemia secundaria en adultos: estudio de cohorte retrospectiva

Background and objective: IgG replacement therapy (IgG-RT) has radically changed the clinical evolution of primary immunodeficiencies, yet the information regarding secondary hypogammaglobulinemia (SHG) is insufficient or conflicting. We aim to describe clinical features, evolution and treatment of SHG patients in our center. Methods: Dynamic retrospective cohort between January 2001 and July 2021 of adults with gamma globulin fraction <0.6g/dL in a serum protein electrophoresis and a coincident decrease of IgG levels – with a disease-related SHG or treatment that reduces serum immunoglobulins. Results: We included 1012 patients with SHG with a median follow-up of 5 years (IQR 2–8). Hematological diseases were identified in 95% of the patients and 61% received drugs related to SHG. Sixty five percent had more than one etiological factor associated with SHG. Infectious diseases were present in 69% of the patients, 48% had respiratory infections and 17% had severe infections. There was statistical association between respiratory and severe infections with multiple myeloma (MM), lymphoma and rituximab. MGUS had less infections and death compared with other etiologies. IgG-RT was indicated in 18.7% of the patients and 4.6% received it for more than 6 months with variable intervals. Among the latter group, there was a significant reduction of all-type infections and respiratory infections with IgG-RT (p<0.001), and it was consistent with similar findings in lymphoma, MM and all IgG levels subgroups. Conclusion: SHG was associated with more than one etiological factor and a high frequency of infections. IgG-RT indication was irregular yet still effective. It is relevant to consider IgG levels screening, monitoring and accurate indication of IgG-RT.(AU)

Antecedentes y objetivos: La IgG sustitutiva ha cambiado radicalmente la evolución de las inmunodeficiencias primarias, mientras que la información sobre hipogammaglobulinemia secundaria (HGS) es insuficiente y discordante. El objetivo del estudio es describir las características clínicas, evolución y tratamiento de pacientes con HGS. Métodos: Cohorte retrospectiva dinámica entre enero de 2001 y julio de 2021 de adultos con proteinograma y fracción de gammaglobulina <0,6g/dL y dosaje disminuido de IgG, con enfermedad o tratamiento que produzcan HGS. Resultados: Se incluyó a 1.012 pacientes con HGS con una mediana de seguimiento de 5 años (IIC 2-8). El 95% tenía enfermedad hematológica y el 61% recibió fármacos asociados a HGS. El 65% tenía más de un factor etiológico asociado con HGS. El 69% presentó infecciones de cualquier tipo, el 48% infecciones respiratorias y el 17%, infecciones graves. Hubo asociación significativa entre infecciones respiratorias y graves entre los subgrupos de mieloma múltiple, linfoma y rituximab. Los pacientes con MGUS tuvieron menor frecuencia de infecciones y muerte comparados con otros factores etiológicos. El 18,7% de los pacientes recibió IgG sustitutiva y el 4,6% de forma crónica, con intervalos variables. Los últimos tuvieron disminución significativa de infecciones de cualquier tipo e infecciones respiratorias con IgG sustitutiva (p<0,001), que se mantuvo en los subgrupos con mieloma múltiple, linfoma y todos los niveles de IgG. Conclusión: La HGS asoció más de un factor etiológico y alta frecuencia de infecciones. La indicación de IgG sustitutiva fue irregular, pero, aún así, efectiva. Se plantea considerar el dosaje de inmunoglobulinas, monitoreo y la adecuada indicación de IgG sustitutiva.(AU)

Detección incidental de niveles de vitamina B12 elevados y su relación con neoplasias / Incidental detection of raised serum levels of vitaminB12 and its association with neoplasms

Antecedentes y objetivo Los niveles elevados de vitaminaB12 se han asociado a enfermedades oncohematológicas. Sin embargo, se desconoce la relevancia de su detección incidental en sujetos sin un diagnóstico previo de cáncer. El objetivo de este estudio es evaluar la relación de la hipercobalaminemia y el diagnóstico de un proceso tumoral y establecer los factores de riesgo. Material y métodos Estudio observacional retrospectivo de una cohorte de pacientes con hipercobalaminemia. Se comparó la incidencia de neoplasias con una cohorte de pacientes con vitaminaB12<1.000pg/ml. Resultados Se seleccionaron 4.800 sujetos con determinaciones de vitaminaB12: 345 (7,1%) presentaban niveles >1.000pg/ml. Se excluyeron 68 (28,4%) por administración exógena, 12 (5%) por datos insuficientes y 15 (3%) por una neoplasia activa, seleccionando 250 pacientes; mediana de seguimiento: 22 (RIQ: 12-39) meses. Se detectó: hepatopatía 59 (23,6%), 44 (18,2%) presentaron cáncer de órgano sólido y 17 (7,1%), hemopatía maligna. El tiempo medio desde la detección de hipercobalaminemia al diagnóstico fue de 10meses. La mediana hasta el diagnóstico fue mayor en el grupo de vitaminaB12 elevada (13 vs 51meses; p<0,001). La hipercobalaminemia (HR_ 11,8; IC95: 2,8-49,6; p=0,001) y el tabaquismo (HR: 4,0; IC95%: 2,15-7,59; p<0,001) resultaron predictores independientes. Conclusiones La detección incidental de niveles séricos de vitaminaB12 >1.000pg/ml es elevada. El diagnóstico de neoplasia órgano sólido y hematológica es frecuente durante el año siguiente de seguimiento, siendo la hipercobalaminemia y el tabaquismo factores predictores de un mayor riesgo de cáncer. (AU)

Background and objective Elevated serum levels of vitaminB12 have been associated with oncohematological diseases. However, the relevance of its incidental detection in subjects without a previous diagnosis of cancer is unknown. The aim of this study was to evaluate the relationship between incidental hypercobalaminemia (vitaminB12 >1000pg/mL) and the diagnosis of a tumor process in patients without a diagnosis and to establish the risk factors. Material and methods Retrospective observational study of a cohort of patients with hypercobalaminemia. The incidence of neoplasms was compared with a cohort of patients with vitaminB12 levels <1000pg/mL. Results Vitamin B12 determinations of 4800 subjects were selected. Of them, 345 (7.1%) had levels >1000pg/ml; 68 (28.4%) were excluded due to exogenous administration, 12 (5%) due to insufficient data, and 15 (3%) due to having an active neoplasia, selecting 250 patients, with a median follow-up of 22 (IQR: 12-39) months. Structural liver disease was detected in 59 (23.6%). 18.2% (44 patients) had solid organ cancer and 17 (7.1%) had malignant hemopathy. The average time from the detection of hypercobalaminemia to the diagnosis of cancer was about 10months. The median until the diagnosis of neoplasia was higher in the high vitaminB12 group (13 vs 51months; P<.001). Hypercobalaminemia (HR: 11.8; 95%CI: 2.8-49.6; P=.001) and smoking (HR: 4.0; 95%CI: 2.15-7.59; P<.001) were independent predictors of neoplasia in the multivariate analysis. Conclusions Incidental detection of serum vitaminB12 levels >1000pg/ml is high in the population. The diagnosis of solid organ and hematological neoplasia is frequent during the following year of follow-up, with hypercobalaminemia and smoking being predictors of a higher risk of cancer. (AU)

Detección incidental de niveles de vitamina B12 elevados y su relación con neoplasias / Incidental detection of raised serum levels of vitaminB12 and its association with neoplasms

Antecedentes y objetivo Los niveles elevados de vitaminaB12 se han asociado a enfermedades oncohematológicas. Sin embargo, se desconoce la relevancia de su detección incidental en sujetos sin un diagnóstico previo de cáncer. El objetivo de este estudio es evaluar la relación de la hipercobalaminemia y el diagnóstico de un proceso tumoral y establecer los factores de riesgo. Material y métodos Estudio observacional retrospectivo de una cohorte de pacientes con hipercobalaminemia. Se comparó la incidencia de neoplasias con una cohorte de pacientes con vitaminaB12<1.000pg/ml. Resultados Se seleccionaron 4.800 sujetos con determinaciones de vitaminaB12: 345 (7,1%) presentaban niveles >1.000pg/ml. Se excluyeron 68 (28,4%) por administración exógena, 12 (5%) por datos insuficientes y 15 (3%) por una neoplasia activa, seleccionando 250 pacientes; mediana de seguimiento: 22 (RIQ: 12-39) meses. Se detectó: hepatopatía 59 (23,6%), 44 (18,2%) presentaron cáncer de órgano sólido y 17 (7,1%), hemopatía maligna. El tiempo medio desde la detección de hipercobalaminemia al diagnóstico fue de 10meses. La mediana hasta el diagnóstico fue mayor en el grupo de vitaminaB12 elevada (13 vs 51meses; p<0,001). La hipercobalaminemia (HR_ 11,8; IC95: 2,8-49,6; p=0,001) y el tabaquismo (HR: 4,0; IC95%: 2,15-7,59; p<0,001) resultaron predictores independientes. Conclusiones La detección incidental de niveles séricos de vitaminaB12 >1.000pg/ml es elevada. El diagnóstico de neoplasia órgano sólido y hematológica es frecuente durante el año siguiente de seguimiento, siendo la hipercobalaminemia y el tabaquismo factores predictores de un mayor riesgo de cáncer. (AU)

Background and objective Elevated serum levels of vitaminB12 have been associated with oncohematological diseases. However, the relevance of its incidental detection in subjects without a previous diagnosis of cancer is unknown. The aim of this study was to evaluate the relationship between incidental hypercobalaminemia (vitaminB12 >1000pg/mL) and the diagnosis of a tumor process in patients without a diagnosis and to establish the risk factors. Material and methods Retrospective observational study of a cohort of patients with hypercobalaminemia. The incidence of neoplasms was compared with a cohort of patients with vitaminB12 levels <1000pg/mL. Results Vitamin B12 determinations of 4800 subjects were selected. Of them, 345 (7.1%) had levels >1000pg/ml; 68 (28.4%) were excluded due to exogenous administration, 12 (5%) due to insufficient data, and 15 (3%) due to having an active neoplasia, selecting 250 patients, with a median follow-up of 22 (IQR: 12-39) months. Structural liver disease was detected in 59 (23.6%). 18.2% (44 patients) had solid organ cancer and 17 (7.1%) had malignant hemopathy. The average time from the detection of hypercobalaminemia to the diagnosis of cancer was about 10months. The median until the diagnosis of neoplasia was higher in the high vitaminB12 group (13 vs 51months; P<.001). Hypercobalaminemia (HR: 11.8; 95%CI: 2.8-49.6; P=.001) and smoking (HR: 4.0; 95%CI: 2.15-7.59; P<.001) were independent predictors of neoplasia in the multivariate analysis. Conclusions Incidental detection of serum vitaminB12 levels >1000pg/ml is high in the population. The diagnosis of solid organ and hematological neoplasia is frequent during the following year of follow-up, with hypercobalaminemia and smoking being predictors of a higher risk of cancer. (AU)

Recomendaciones SER sobre la gestión de riesgo del tratamiento con FAME biológicos o sintéticos dirigidos en pacientes con artritis reumatoide / Recommendations by the Spanish Society of Rheumatology on risk management of biological treatment and JAK inhibitors in patients with rheumatoid arthritis

Objetivo: Elaborar recomendaciones basadas en la evidencia disponible y el consenso de expertos, para la gestión del riesgo del tratamiento biológico y los inhibidores de las JAK en pacientes con artritis reumatoide. Métodos: Se identificaron preguntas clínicas de investigación relevantes para el objetivo del documento. Estas preguntas fueron reformuladas en formato PICO (paciente, intervención, comparación, outcome o desenlace) por un panel de expertos, seleccionados en base a su experiencia en el área. Se realizó una revisión sistemática de la evidencia, graduándose de acuerdo a los criterios GRADE (Grading of Recommendations Assessment, Development, and Evaluation). A continuación, se formularon las recomendaciones específicas. Resultados: Se propusieron por el panel de expertos 6preguntas PICO en base a su relevancia clínica y a la existencia de información reciente referentes al riesgo de aparición de infecciones graves, el riesgo de reactivación del virus de la hepatitisB, el riesgo de reactivación del virus varicela-zoster, el riesgo de aparición de cáncer de piel (melanoma y no melanoma) o hematológico, el riesgo de aparición de enfermedad tromboembólica y el riesgo de progresión del virus del papiloma humano. Se formularon un total de 29 recomendaciones, estructuradas por pregunta, basadas en la evidencia encontrada y el consenso de los expertos. Conclusiones: Se presentan las recomendaciones SER sobre la gestión del riesgo del tratamiento con terapias biológicas e inhibidores de las JAK en la artritis reumatoide.(AU)

Objective: To present recommendations based on the available evidence and the consensus of experts, for risk management of biological treatment and JAK inhibitors in patients with rheumatoid arthritis. Methods: Clinical research questions relevant to the purpose of the document were identified. These questions were reformulated in PICO format (patient, intervention, comparison, outcome) by a panel of experts, selected based on their experience in the area. A systematic review of the evidence was carried out, grading according to the GRADE criteria (Grading of Recommendations Assessment, Development, and Evaluation). Specific recommendations were then formulated. Results: Six PICO questions were proposed by the panel of experts based on their clinical relevance and the existence of recent information regarding the risk of occurrence of serious infections, the risk of reactivation of the hepatitisB virus, the risk of reactivation of the virus varicella-zoster, the risk of appearance of skin (melanoma and non-melanoma) or hematological cancer, the risk of appearance of thromboembolic disease and the risk of progression of the human papilloma virus. A total of 29 recommendations were formulated, structured by question, based on the evidence found and the consensus of the experts. Conclusions: The SER recommendations on risk management of treatment with biologic therapies and JAK inhibitors in rheumatoid arthritis are presented.(AU)

Systemic Mastocytosis with Associated Hematological Neoplasms. Diagnostic features and unique response pattern to tyrosine kinase inhibitors and allo-bone marrow transplantation therapy / Mastocitosis sistémica con neoplasias hematológicas asociadas. Características diagnósticas y patrón de respuesta único a los inhibidores de la tirosina cinasa y al tratamiento de trasplante de médula ósea

Systemic Mastocytosis is a clonal proliferation of mast cells; in a significant fraction of cases it is associated with another concurrent hematological neoplasm. Molecular analysis of KIT mutations and other associated genetic alterations suggest a common origin in the stem cell compartment. Mast cell infiltration patterns in bone marrow biopsy may be subtle in cases associated with t (8;21) AML. Here we report three cases of clonally related SM-AHN, two cases with SM-CMML and one case with SM- t (8;21) AML. We describe in detail the bone marrow infiltration pattern at diagnosis and during the course of treatment with allogeneic stem cell transplant and novel TK inhibitors, showing the unique dynamics of mast cell clearance after therapy.(AU)

La mastocitosis sistémica es una proliferación clonal de mastocitos que puede asociarse con otra neoplasia hematológica concurrente en una fracción significativa de los casos. El análisis molecular de mutaciones de KIT y otras alteraciones genéticas asociadas indican un origen común en el compartimento de células madre. Los patrones de infiltración de mastocitos en la biopsia de médula ósea pueden ser sutiles en los casos asociados con AML t (8; 21). Aquí se describen 3 casos de MS-NHA, 2 casos con MS-LMMC y un caso con MS-t (8; 21) LMA. Describimos en detalle el patrón de infiltración de la médula ósea en el momento del diagnóstico y durante el curso del tratamiento con alotrasplante de células madre y nuevos inhibidores de TK, mostrando la dinámica de la depuración de mastocitos después de la terapia.(AU)

¿Enfermedad o reacción pulmonar intersticial? Investigar hasta encontrar el origen / Interstitial lung disease or reaction? Investigate until you find the source

La neumonía organizativa es un patrón histológico que puede observarse asociado o reactivo en diferentes patologías.Presentamos el caso de una mujer de 67 años con diagnóstico inicial de neumonía organizada criptogénica, sin respuestaa los glucocorticoides y autoinmunidad negativa. Finalmente, la neumonía organizada resultó ser secundaria a neoplasiahematológica. En estos casos es importante insistir en la anamnesis y realizar los procedimientos necesarios para conseguirel diagnóstico definitivo.(AU)

Organizational pneumonia is a histological pattern that can be observed associated or reactive in different pathologies. Wepresent the case of a 67-year-old woman with an initial diagnosis of organized pneumonia, no response to glucocorticoidsand negative autoimmunity. Finally, organized pneumonia was found to be secondary to hematologic malignancy. In thesecases, it is important to insist on the anamnesis and perform the necessary procedures to achieve the definitive diagnosis.(AU)

Características de los pacientes con neoplasias hematológicas y con tumores sólidos atendidos por un equipo de soporte paliativo hospitalario / Characteristics of patients with hematological and solid tumors malignancies attended by a hospital palliative care service

Antecedentes y objetivo: El interés por la atención paliativa en pacientes con neoplasias hematológicas está aumentando. Nuestro objetivo es describir las características de pacientes oncológicos valorados por un equipo de soporte paliativo en un hospital terciario y analizar las diferencias entre pacientes hematológicos y con tumores sólidos. Método: Estudio observacional descriptivo longitudinal retrospectivo con una cohorte de pacientes hospitalizados con enfermedad oncológica (hematológica o tumor sólido) valorados por equipo de soporte paliativo hospitalario. Comparamos variables clínicas, asistenciales y de supervivencia. El análisis de datos se realizó con la versión 15 del programa SPSS. Resultados: De enero de 2015 a diciembre de 2018 se valoraron 1025 pacientes oncológicos (10,8 % hematológicos, 89,2 % sólidos). No se encontraron diferencias en situación funcional medida por la Palliative Performance Scale, presentación de síntoma principal, porcentaje de pacientes con dolor, tiempo de seguimiento ni en porcentaje de fallecidos en el ingreso en que fueron valorados. El paciente hematológico, comparado con el oncológico, tiene menos tratamiento opioide pautado (43 vs. 53 %; p = 0,035), es seguido con más frecuencia por recurso paliativo hospitalario que domiciliario (46,55 vs. 29,44 % el primero; 15,5 vs. 33,06 % el segundo; p = 0,001 en distribución) y fallece más en hospital (82,9 vs. 65,5 %; p = 0,024). Conclusiones: Los pacientes con neoplasia hematológica presentan una carga sintomática similar a los pacientes con tumor sólido. Es importante identificar mejor sus necesidades para que puedan beneficiarse, como se ha demostrado con los pacientes oncológicos, de la atención integrada junto a los servicios de hematología con modelos de intervención acordes a sus necesidades y las trayectorias específicas de las enfermedades hematológicas. (AU)

Background and objective: Interest in palliative care for patients with hematologic malignancies is increasing. Our goal is to describe the features of cancer patients evaluated by a supportive and palliative care service in a tertiary referral hospital, and to analyze the differences between patients with hematological malignancies and solid tumors. Method: A retrospective longitudinal descriptive observational study was carried out in a cohort of hospitalized patients with oncological diseases (hematological or solid tumor) evaluated by a palliative care service. We compared clinical, healthcare and survival variables between both groups. The analysis was performed using the SPSS v.15 package. Results: From January 2015 to December 2018, 1025 cancer patients were evaluated (10.8 % hematological tumor, 89.2 % solid tumors). No differences were found in functional status as measured by the Palliative Performance Scale, presentation of main symptom, percentage of patients with pain, time of follow-up, or percentage of deaths on admission to the evaluation. The hematological patient, compared to the oncological one, has less prescribed opioid treatment (43 % vs 53 %, p = 0.035), received greater hospital palliative care rather than home-based care (46.55 % vs 29.44 % the former and 15.5 % vs 33.06 % the latter, p = 0.001), and dies more frequently in a hospital (82.9 % vs. 65.5 %, p = 0.024). Conclusions: Patients with hematological malignancies present a symptomatic burden similar to that of those with solid tumors. It is important to better identify their needs so that they can benefit, as has been demonstrated with cancer patients, from integrated care together with hematology services using intervention models according to their needs and specific disease trajectories. (AU)

The need for evolution in the management of febrile neutropenia in pediatric cancer: TRIIO KIDS update

New treatments have increased the life expectancy of pediatric patients diagnosed with malignant hematological diseases, often at the cost of protracting their immunocompromised state in the form of prolonged neutropenia. This neutropenic state favors the development of bacterial and fungal infections. Moreover, recent years have seen a series of changes in the epidemiology of fungal and Clostridium infections. These changes necessitate adaptations to the management of pediatric patients with febrile neutropenia, who are at risk of further increases in already high rates of morbidity and mortality. This article discusses the current bases for the management of febrile neutropenia and associated emerging fungal infections, as well as the epidemiology, diagnosis, and treatment of Clostridioides difficile in pediatric patients diagnosed with malignant hematological diseases (AU)

Impact of the COVID-19 pandemic on the diagnosis and treatment of onco-hematologic patients: a discussion paper / Impacto de la pandemia de COVID-19 en el diagnóstico y tratamiento de los pacientes onco-hematológicos: un documento de opinión

We do not know the precise figure for solid organ tumors diagnosed each year in Spain and it is therefore difficult to calculate whether there has been a decrease in cancer diagnoses as a consequence of the pandemic. Some indirect data suggest that the pandemic has worsened the stage at which some non-hematological neoplasms are diagnosed. Despite the lack of robust evidence, oncology patients seem more likely to have a poor outcome when they contract COVID-19. The antibody response to infection in cancer patients will be fundamentally conditioned by the type of neoplasia present, the treatment received and the time of its administration.In patients with hematological malignancies, the incidence of infection is probably similar or lower than in the general population, due to the better protective measures adopted by the patients and their environment. The severity and mortality of COVID-19 in patients with hematologic malignancies is clearly higher than the general population. Since the immune response to vaccination in hematologic patients is generally worse than in comparable populations, alternative methods of prevention must be established in these patients, as well as actions for earlier diagnosis and treatment.Campaigns for the early diagnosis of malignant neoplasms must be urgently resumed, post-COVID manifestations should be monitored, collaboration with patient associations is indisputable and it is urgent to draw the right conclusions to improve our preparedness to fight against possible future catastrophes. (AU)

No conocemos con precisión la cifra nacional de tumores sólidos diagnosticados en España anualmente y por tanto se hace difícil calcular si ha habido una disminución de diagnósticos de cáncer como consecuencia de la pandemia. Algunos datos indirectos permiten sospechar que la pandemia ha empeorado el estadio en que se diagnostican algunas neoplasias no hematológicas. A pesar de no existir una evidencia robusta, los pacientes oncológicos presentan una mayor tendencia a tener una mala evolución cuando contraen COVID-19. La respuesta de anticuerpos frente a la infección en pacientes con cáncer va a estar condicionada fundamentalmente por el tipo de neoplasia presente, el tratamiento recibido y el momento de su administración.En pacientes con hemopatías malignas la incidencia de infección es probablemente similar o inferior a la de la población general, debido a las mejores medidas de protección adoptadas por los pacientes y su entorno. La gravedad y letalidad de la COVID-19 en pacientes con hemopatías malignas es claramente más elevada que en la población general. Dado que la respuesta inmune a la vacunación es peor que en poblaciones comparables, hay que establecer métodos alternativos de prevención en estos pacientes, así como planes de diagnóstico y tratamiento precoces.Hay que retomar las campañas de diagnóstico precoz de neoplasias malignas con urgencia, vigilar las manifestaciones post-COVID, colaborar con las asociaciones de pacientes y hacer planes urgentes para hacer frente con más eficiencia a potenciales catástrofes futuras. (AU)

Outcomes and impact of multidisciplinary team care on immunologic and hemato-oncologic pediatric patients

Introduction: Immunologic and hemato-oncologic disorders in the pediatric population represent an interrelated and complex group of conditions whose approach, diagnosis, and management could be difficult. Multidisciplinary teams have been proved beneficial in treating such complexities. Methods: We conducted a retrospective observational study at a tertiary hospital in Madrid, Spain, which is a pediatric immunology and onco-hematology referral center. We included all patients of multidisciplinary outpatient consultation, comprising a working group of pediatric oncohematologists and immunologists, between April 2016 and December 2019. Epidemiologic, clinical, and laboratory data were collected. We analyzed these data and established a relationship between age and findings of final diagnosis as well as variance on diagnoses prior to their multidisciplinary assessment and number of visits to the consultation. Results: In all, 93 children and adolescents were included in this study. Laboratory abnormalities were the most frequent reason for being referred to our unit (87.2%); 78% of children had a previously diagnosed comorbidity. Before starting follow-up in the multidisciplinary consultation, 14% of patients were diagnosed, and after the study by the multidisciplinary team, the final diagnosis was reached in 58.1% of patients. No correlation was discovered between final diagnosis and gender (P = 0.29), age (biserial correlation coefficient, r = 0.036, P = 0.70), and number of visits (P = 0.07). Conclusion: A multidisciplinary approach to immunologic, hematologic, and oncologic pediatric diseases is feasible. It can be a powerful and useful tool for diagnosis and treatment, especially in complex pediatric patients (AU)

Long-term treatment with nebulized colistin in oncological patients with recurrent respiratory infections

Objectives: To assess the efficacy of long-term treatment with nebulized colistin in reducing the number of respiratory infections, emergency consultations and hospitalizations in oncological patients.MethodsA retrospective, observational, single-centre study including patients with solid or haematologic malignancies, or pulmonary GVHD after HSTC who received treatment with nebulized colistin for at least six-months to prevent recurrent respiratory infections (July 2010 to June 2017).ResultsTwelve patients were included (median age: 54.4, range: 23–85), 7 with solid malignancies and 5 with haematologic malignancies (2 with pulmonary GVHD). Pseudomonas aeruginosa was the most frequent microorganism in sputum cultures (11/12 patients), all strains were susceptible to colistin. There was a statistically significant reduction (p=0.01) in respiratory infections in the six-month period after starting colistin (median: 1, range: 0–4) compared to the six-month period before (median: 4, range: 1–8). There was also a reduction in emergency consultations (precolistin: median: 1.50, range: 0–3; postcolistin: median: 0, range: 0–3) and hospitalizations (precolistin: median: 1.50, range: 0–3; postcolistin: median: 0, range: 0–3) due to respiratory infections. No colistin-resistant strains were identified.ConclusionsLong-term treatment with nebulized colistin may be useful to reduce the number of exacerbations in oncological patients with recurrent respiratory infections. (AU)

Objetivos: Evaluar la eficacia de un tratamiento prolongado con colistina nebulizada para reducir el número de infecciones respiratorias, consultas en Urgencias y hospitalizaciones en pacientes oncológicos.MétodosEstudio retrospectivo, observacional y unicéntrico en pacientes con neoplasias sólidas o hematológicas o EICR pulmonar tras TPH tratados con colistina nebulizada al menos 6 meses para prevenir infecciones respiratorias recurrentes (julio del 2010-junio del 2017).ResultadosSe incluyó a 12 pacientes (edad mediana 54,4, rango: 23-85), 7 con cáncer sólido y 5 con neoplasias hematológicas (2 con EICR pulmonar). El microorganismo aislado más frecuentemente en esputos fue Pseudomonasaeruginosa (11/12 pacientes); todas las cepas fueron colistina-sensibles. Se evidenciaron una reducción estadísticamente significativa (p = 0,01) de las infecciones respiratorias en los 6 meses tras iniciar colistina (mediana: 1, rango: 0-4) comparado con los 6 meses previos (mediana: 4, rango: 1-8), y una reducción del número de visitas a Urgencias (precolistina: mediana: 1,50, rango: 0-3; postcolistina: mediana: 0, rango: 0-3) y hospitalizaciones (precolistina: mediana: 1,50, rango: 0-3; postcolistina: mediana: 0, rango: 0-3) por infección respiratoria. No se detectaron cepas resistentes a colistina.ConclusionesUn tratamiento prolongado con colistina nebulizada puede ser útil para reducir el número de exacerbaciones en pacientes oncológicos con infecciones respiratorias recurrentes. (AU)

Infección del sistema nervioso central por Bacillus cereus: descripción de un caso y revisión de la bibliografía / Central nervous system infection by Bacillus cereus: a case report and literature review

Introducción: Bacillus cereus es un patógeno ubicuo que, habitualmente, produce síntomas gastrointestinales autolimitados. Sin embargo, en pacientes susceptibles, puede dar lugar a infecciones del sistema nervioso central potencialmente mortales. Desarrollo: Presentamos el caso de un varón de 10 años en tratamiento quimioterápico por leucemia linfoblástica aguda. Durante el período de inducción desarrolló un absceso cerebral por B. cereus que fue diagnosticado mediante pruebas de imagen y detección directa en el líquido cefalorraquídeo. Su evolución fue favorable con tratamiento antibiótico. Conclusiones: Hasta ahora se han descrito en la bibliografía otros 26 casos de infección del sistema nervioso central por B. cereus, que, además de ser infrecuentes, suponen un reto diagnóstico. Sin embargo, en los recién nacidos prematuros, en pacientes con neoplasias hematológicas o con antecedentes de cirugía del sistema nervioso central, debe establecerse una sospecha temprana para iniciar un tratamiento antibiótico adecuado que mejore el pronóstico.(AU)

Introduction: Bacillus cereus is a ubiquitous pathogen that usually produces self-limiting gastrointestinal symptoms. However, in susceptible patients, it can lead to central nervous system infections which are potentially fatal. Development: We present the case of a 10-year-old male under chemotherapy treatment for acute lymphoblastic leukemia. During the induction period he developed a brain abscess due to B. cereus which was diagnosed through imaging tests and direct detection in the cerebrospinal fluid. His evolution was favorable with antibiotic treatment. Conclusions: So far, 26 other cases of central nervous system infections due to B. cereus have been described in literature, and besides being infrequent, they are a diagnostic challenge. However, in preterm infants, patients with hematological malignancies or central nervous system surgery, early suspicion should be established to start an appropriate antibiotic treatment and improve prognosis.(AU)

A strategic reflection for the management and implementation of CAR-T therapy in Spain: an expert consensus paper

CAR-T cell therapy represents a therapeutic revolution in the prognosis and treatment of patients with certain types of hematological cancer. However, they also pose new challenges in the healthcare, regulatory and financial fields. The aim of the RET-A project was to undertake a strategic reflection on the management of CAR-T therapies within the Spanish National Health System, to agree on recommendations that will help to better deal with the new context introduced by these cell therapies in the present and in the future. This think tank involved 40 key agents and opinion leaders. The experts identified three great challenges for implementing advanced therapies in Spain: therapeutic individualisation, with a multidisciplinary approach; acceleration of access times, by minimizing bureaucracy; and increase in the number of centers qualified to manage the CAR-T therapies in the NHS. The experts agreed on the ideal criteria for designating those qualified centers. They also agreed on a comprehensive CAR-T care pathway with the timings and roles which would ideally be involved in each part of the process.

Prevention of venous thromboembolism in hematologic neoplasms: an expert consensus from SEHH–SETH

Venous thromboembolism (VTE) is a serious complication in hematologic neoplasms, so finding adequate prevention strategies is an urgent requirement. However, prospective studies with large enough cohorts are scarce, limiting the development of evidence-based thromboprophylaxis guidelines. The present position paper is addressed to all hematologists treating patients affected by hematologic neoplasms with the aim to provide clinicians with a useful tool for the prevention of VTE.

Polineuropatía periférica sensitivo-motora paraneoplásica asociada a anti-Ri: a propósito de un caso / Paraneoplastic sensory-motor peripheral polyneuropathy associated with anti-Ri: A case report

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Desesperanza y su relación con el apoyo social percibido en pacientes peruanos con neoplasias hematológicas / Hopelessness and its relationship with perceived social support in Peruvian patients with hematological neoplasms

Objetivo: El propósito del presente estudio fue determinar la relación significativa entre el apoyo social y la desesperanza en pacientes peruanos con neoplasias hematológicas que se encuentren en alguna de las fases del tratamiento. Método: El estudio fue de tipo descriptivo-correlacional. La muestra estuvo conformada por 110 pacientes hematooncológicos de ambos sexos, con edades entre 16 a 25 años. Los instrumentos utilizados fueron la Escala Multidimensional de Apoyo Social Percibido (MSPSS) y la Escala de Desesperanza de Beck (BHS). Resultados: Se reportan niveles altos de apoyo social percibido (81%) y niveles normales de desesperanza (82%) en los pacientes hematooncológicos. El apoyo social percibido se correlaciona inversa y significativamente con la desesperanza, aunque en magnitudes bajas a moderadas. Cabe destacar que la dimensión Expectativa de desgracia en el futuro, no se correlaciona significativamente con el apoyo social percibido y sus dimensiones. Igualmente, la dimensión Creencias que los acontecimientos negativos son durables, no se correlaciona con la dimensión Apoyo de familia. Por otro lado, se encontró diferencias significativas del apoyo social percibido con el sexo y la religión, asimismo, la desesperanza con la edad, tiempo de diagnóstico y fase de tratamiento. Conclusión: El apoyo social percibido se relaciona con la desesperanza en pacientes hematooncológicos peruanos. Por tanto, se corrobora que el apoyo social que recibe el paciente ante la desesperanza de una enfermedad hematooncológica, sirve como soporte y tolerancia de dicha enfermedad y sus efectos durante el tratamiento (AU)

Objective: The aim of this study was to determine the significant relationship between social support and hopelessness in Peruvian patients with hematological neoplasms who are in any of the treatment phases. Method: The study was descriptive-correlational. The sample consisted of 110 hemato-oncological patients of both sexes, aged between 16 and 25 years. The instruments used were the Multidimensional Scale of Perceived Social Support (MSPSS) and the Beck Hopelessness Scale (BHS). Results: High levels of perceived social support (81%) and normal levels of hopelessness (82%) are reported in hemato-oncological patients. Perceived social support is inversely and significantly correlated with hopelessness, although in low to moderate magnitudes. However, the dimension Expectation of misfortune in the future, does not correlate significantly with the perceived social support and its dimensions. Likewise, the Beliefs that negative events are durable dimension did not correlate with the dimension Family support. On the other hand, significant differences were found in perceived social support with sex and religion, as well as hopelessness with age, time of diagnosis and treatment phase. Conclusion: The perceived social support is related to the hopelessness of Peruvian hemato-oncological patients. Therefore, it is corroborated that the social support that the patient receives when faced with the hopelessness of a hemato-oncological disease, serves as support and tolerance of said disease and its effects during treatment (AU)