Health-related quality of life and utility outcomes with selinexor in relapsed/refractory diffuse large B-cell lymphoma.

Aim: Evaluate health-related quality of life (HRQoL) and health utility impact of single-agent selinexor in heavily pretreated patients with relapsed/refractory diffuse large B-cell lymphoma. Patients & methods: Functional Assessment of Cancer Therapy (FACT) - Lymphoma and EuroQoL five-dimensions five-levels data collected in the single-arm Phase IIb trial SADAL (NCT02227251) were analyzed with mixed-effects models. Results: Treatment responders maintained higher FACT - Lymphoma (p ≤ 0.05), FACT - General (p < 0.05) and EuroQoL five-dimensions five-levels index scores (p < 0.001) beginning in cycle 3. The estimated difference in health state utilities for treatment response and progressive disease was both statistically significant and clinically meaningful (mean difference: 0.07; p = 0.001). Conclusion: In patients with relapsed/refractory diffuse large B-cell lymphoma, objective response to selinexor was associated with HRQoL maintenance, reduction in disease-related HRQoL decrements and higher health utilities.

Lay abstract This work examined quality of life (QoL) among patients with relapsed/refractory diffuse large B-cell lymphoma with two to five prior therapies who received single-agent selinexor in the SADAL clinical trial. Analysis of patient-reported Functional Assessment of Cancer Therapy ­ Lymphoma and EuroQoL five-dimensions five-levels data showed that patients who had objective clinical response to selinexor maintained their QoL over the course of treatment. Grade ≥3 adverse events and serious adverse events were not associated with clinically meaningful negative QoL impacts. Clinical trial registration: NCT02227251 (ClinicalTrials.gov).

Tomografía por emisión de positrones en linfoma cerebral primario: implicación diagnóstica y pronóstica / Positron emission tomography in primary brain lymphoma: Diagnostic and prognostic significance

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Evaluación del impacto de cartas de condolencia en familiares en duelo / Assessment of the impact of condolence letters on bereaved families

OBJETIVOS: Evaluar el impacto emocional y la utilidad percibida de las cartas de condolencia en familiares de pacientes fallecidos en los servicios de nefrología y hematología. MÉTODO: Estudio prospectivo con una única medida en forma de entrevista telefónica semiestructurada. Cuestionario elaborado ad hoc. Las variables se evaluaban de forma independiente. La muestra consta 102 muertes y 82 dolientes. RESULTADOS: Un 15% de la muestra refiere haber sentido malestar al recibir la carta y el 100% de la muestra sintió bienestar (categorías no excluyentes), con emociones como gratitud (42,5%), alegría (40%), orgullo (10%) y sorpresa (7,5%). El 96,3% compartió la carta con el entorno. El 98,8% refiere que la carta le ha sido útil. Los pacientes renales son mayores en el momento del fallecimiento (U = 599,00; p < 0,001), han pasado más años en seguimiento por el servicio (χ2 = 19,40; p < 0,001) y responden con mayor frecuencia a la carta de condolencia a través de llamadas telefónicas, visitas al servicio o tarjetas postales (χ2 = 5,30; p < 0,001). No se han encontrado diferencias estadísticamente significativas en relación con los resultados del impacto de la carta entre los familiares de los 2 servicios. DISCUSIÓN: El impacto de la carta ha producido bienestar en todos los familiares y malestar en un 15% de la muestra. Se impone la necesidad de considerar en el duelo emociones opuestas pero no incompatibles. Conocer esta ambivalencia propia de los procesos de pérdida ayudará a la normalización de la misma

OBJECTIVES: To assess the emotional impact and perceived usefulness of condolence letters in families of patients who died in the hospital Nephrology and Haematology departments. Method: A prospective study was conducted with a single one measure in the form of a semistructured telephone interview. The questionnaire was constructed ad hoc. Variables were assessed independently. The sample consisted of 102 deceased and 82 grievers. RESULTS: Of the total responses 15% related to have felt discomfort on receiving the letter and100% of the sample (non-exclusive percentages) were comforted, reporting emotions such as, gratitude (42.5%) happiness (40%), pride (10%), and surprise (7.5%). The large majority (96.3%)shared the letter with those around them, and 98.8% said that the letter was useful. The patients with kidney disease were older at time of death (U = 599.00; P=.008), were attended for more years in the department (2 = 19. ,40; P<.001) and their families responded more frequently to the condolence letter through phone calls, visits to the department, or by post card (2 = 5.30;P<.021). The difference between the results of the impact of the letter on both departments was not statistically significant. DISCUSSION: The impact of the letter gave comfort to all the families, and discomfort in 15%of the sample. It shows the need to consider the opposite, but not incompatible emotions, in bereavement. Awareness of this ambivalence, which is typical in the grieving process, will help to normalise it

Impacto clínico de la cuantificación de la carga mutacional de JAK2V617F en pacientes con neoplasias mieloproliferativas crónicas Ph negativas clásicas / Clinical significance of the quantification of JAK2V617F allele burden in classical Ph-negative myeloproliferative neoplasms

Fundamento y objetivo: La identificación de la mutación V617F del gen JAK2 en pacientes diagnosticados de neoplasias mieloproliferativas crónicas (NMPC) es fundamental en el cribado diagnóstico. Nuestro objetivo fue investigar la asociación entre la cuantificación de la mutación V617F (carga alélica JAK2V617F) y el fenotipo clínico al diagnóstico, y definir el papel de la carga alélica en la predicción de complicaciones. Pacientes y métodos: En el Servicio de Hematología del Hospital Universitario La Paz realizamos un estudio observacional retrospectivo (1987-2011) en 114 pacientes diagnosticados de NMPC y análisis de detección de la mutación V617F: 39 policitemias vera (PV), 71 trombocitemias esenciales y 6 mielofibrosis primarias. El porcentaje de alelos mutados fue evaluado en polimorfonucleares de sangre periférica mediante la técnica quantitative real time polymerase chain reaction (qRT-PCR, «reacción en cadena de la polimerasa cuantitativa en tiempo real»). En función de si la carga tumoral se encuentra entre 1-50% o es>50% los pacientes fueron diagnosticados de JAK2mut heterocigoto u homocigoto, respectivamente. Resultados: Se realizó el análisis cuantitativo por qRT-PCR en los 114 pacientes incluidos en el estudio, detectando la mutación V617F en 69 casos y siendo negativa para los 45 pacientes restantes. Encontramos que la presencia de mutación se asocia con la trombosis, y especialmente con la trombosis arterial. Además, y en la serie completa, la carga alélica homocigótica se asocia con diagnóstico de PV, edad avanzada, leucocitosis, mayor hematopoyesis y esplenomegalia. Conclusiones: La detección de la mutación V617F está asociada a una mayor incidencia de trombosis, leucocitosis y esplenomegalia. Su identificación nos permitiría una mejor estratificación pronóstica de los pacientes diagnosticados de NMPC (AU)

Background and objectives: Our study has investigated the presence of the mutation V617F in the JAK2 gene in patients diagnosed with chronic myeloproliferative neoplasms (MPNs). Furthermore, we determined if JAK2 (V617F) allelic burden associates with a specific clinical phenotype and if its quantification can be used as a marker to predict outcome and complications in patients with MPNs.Patients and methods: A retrospective observational study was conducted from 1987 to 2011 in the Haematology Department of La Paz University Hospital. The allelic burden was measured in 114 patients diagnosed with MPNs: 39 polycythemia vera (PV) patients, 71 essential thrombocythaemia patients and 6 primary myelofibrosis patients. The quantitative real-time polymerase chain reaction (qRT-PCR) technology was used to determinate the percentage of mutated alleles in peripheral blood neutrophils. Patients were divided in 2 groups: heterozygous if the result was≤50% of the tested cells, and homozygous if it was positive in>50% of the cells. Results: Sixty-nine patients were positive for the JAK2 mutation and 45 were negative. Among those positive, the mutation was associated with arterial thrombosis. In addition, we demonstrate in the homozygous group that the V617F mutation is associated to PV, advanced age, leukocytosis, marked haematopoiesis and splenomegaly. Conclusions: The presence of V617F mutation is associated with a higher incidence of thrombosis, leukocytosis and splenomegaly. The identification of mutation on the JAK2 gene could help in a better definition of evolution and prognostic stratification of the myeloproliferative disorders (AU)

[Clinical significance of the quantification of JAK2V617F allele burden in classical Ph-negative myeloproliferative neoplasms]. / Impacto clínico de la cuantificación de la carga mutacional de JAK2V617F en pacientes con neoplasias mieloproliferativas crónicas Ph negativas clásicas.

BACKGROUND AND OBJECTIVES: Our study has investigated the presence of the mutation V617F in the JAK2 gene in patients diagnosed with chronic myeloproliferative neoplasms (MPNs). Furthermore, we determined if JAK2 (V617F) allelic burden associates with a specific clinical phenotype and if its quantification can be used as a marker to predict outcome and complications in patients with MPNs. PATIENTS AND METHODS: A retrospective observational study was conducted from 1987 to 2011 in the Haematology Department of La Paz University Hospital. The allelic burden was measured in 114 patients diagnosed with MPNs: 39 polycythemia vera (PV) patients, 71 essential thrombocythaemia patients and 6 primary myelofibrosis patients. The quantitative real-time polymerase chain reaction (qRT-PCR) technology was used to determinate the percentage of mutated alleles in peripheral blood neutrophils. Patients were divided in 2 groups: heterozygous if the result was≤50% of the tested cells, and homozygous if it was positive in>50% of the cells. RESULTS: Sixty-nine patients were positive for the JAK2 mutation and 45 were negative. Among those positive, the mutation was associated with arterial thrombosis. In addition, we demonstrate in the homozygous group that the V617F mutation is associated to PV, advanced age, leukocytosis, marked haematopoiesis and splenomegaly. CONCLUSIONS: The presence of V617F mutation is associated with a higher incidence of thrombosis, leukocytosis and splenomegaly. The identification of mutation on the JAK2 gene could help in a better definition of evolution and prognostic stratification of the myeloproliferative disorders.