T-cell lymphoblastic lymphoma is an uncommon lymphoid neoplasm in adults, although more frequent in children and teenagers, that often affects the mediastinum and bone marrow, requiring intensive ...chemotherapy protocols. Its prognosis is poor if a cure is not achieved with first-line treatments. We present a case report of a 19-year-old man diagnosed with this type of lymphoma due to significant respiratory distress and a mediastinal mass. He received treatment according to the hyper-CVAD regimen, with a complete metabolic response. However, seven months later a new mediastinal growth was observed, leading to salvage treatment with a combination of nelarabine and daratumumab. We observed not only refractoriness, but also leukemization, which prompted consideration of hematopoietic stem cell transplantation. Based on this case, we conducted a review of pharmacological treatment options for refractory or relapsed lymphoblastic lymphoma, as well as the role of radiotherapy in managing mediastinal disease. This case report highlights the limited evidence available regarding later-line treatments, with unusual reports regarding employing our combination of daratumumab and nelarabine, and emphasizes the importance of achieving cures in the first line of treatment.
El desarrollo de derrames pleurales en el postoperatorio de pacientes sometidos a cirugía cardiaca es una complicación frecuente. Se han desarrollado diferentes sistemas de drenaje, entre los cuales, ...aquellos basados en el método Seldinger demuestran mayor seguridad y menor invasividad. La toracocentesis con catéter venoso central supone una maximización de estos aspectos, demostrándose eficaz en la evacuación de derrames pleurales en el contexto del postoperatorio de cirugía cardiaca. Describimos nuestra experiencia incorporando modificaciones técnicas al procedimiento con la utilización de aguja de Tuohy de 18G para la punción.
Pleural effusions are frequent complications in the postoperative period of patients undergoing cardiac surgery. Different drainage systems have been developed, and those based on the Seldinger method demonstrate greater safety and less invasiveness. Thoracentesis with a central venous catheter maximizes these aspects, providing effective evacuation of pleural effusions in the postoperative period of cardiac surgery. We describe our experience adding technical modifications based on puncture with 18G Tuohy needles.
Los injertos de vena safena (IVS) son los conductos más frecuentemente utilizados en la cirugía de revascularización. Sin embargo, su tasa de fallo es del 35-50% a 5-10años, debido en parte a la ...hiperplasia intimal secundaria al estrés parietal. Diferentes exostents han sido desarrollados a fin de paliar este efecto. Se realizó una búsqueda sistemática en Pubmed con diferentes combinaciones de palabras clave para identificar los estudios sobre el uso de exostents. Se realizaron dos metaanálisis, uno basado en la permeabilidad de los injertos y otro sobre el desarrollo de hiperplasia intimal patológica, correspondiéndose con los gradosII-III de la clasificación de Fitzgibbon. Se realizó una puesta al día de la evidencia en torno a los 5exostents disponibles. Se seleccionaron 10 trabajos comparativos controlados para los metaanálisis. La utilización de exostents resultó perjudicial en términos de permeabilidad (RR: 1,67; p=0,03). No obstante, en el análisis de subgrupos, los dispositivos VEST® no mostraron diferencias significativas respecto del grupo control (RR: 1,11; p=0,59). Además, estos dispositivos permitieron mantener un mayor porcentaje de injertos en gradoI de hiperplasia intimal frente a los injertos no soportados (RR: 0,53; p=0,02). Se concluyó que los exostents, globalmente considerados, no mejoran la permeabilidad de los IVS a corto-medio plazo. Con el seguimiento realizado, algunos dispositivos como VEST® han demostrado limitar el desarrollo de hiperplasia intimal, lo cual podría comportar beneficios en cuanto a permeabilidad de los injertos en la prolongación del seguimiento de los trabajos en curso.
Saphenous vein grafts (SVI) are the most common conduits used in revascularization surgery. However, its failure rate is 35-50% at 5-10years, partially due to intimal hyperplasia secondary to shear wall stress. Different exostents have been developed in order to amend this effect. A systematic review was performed in Pubmed with different combinations of keywords to identify all studies about the use of exostents. Two meta-analyses were performed, one based on the patency of the grafts and another focused on the development of pathological intimal hyperplasia, corresponding to gradesII-III of the Fitzgibbon classification. An update of the evidence was carried out regarding the 5 exostents already available. Ten controlled comparative studies were selected for the meta-analyses. The use of exostents was detrimental in terms of patency (RR: 1.67; P=.03). However, in the subgroup analysis, the VEST® devices did not show significant differences compared to the control group (RR: 1.11; P=.59). Furthermore, these devices allowed maintaining more grafts in gradeI of intimal hyperplasia, compared to unsupported grafts (RR: 0.53; P=.02). To conclude, exostents, globally considered, do not improve IVS patency in the mid-short term follow-up. However, some devices such as VEST®, have shown to limit the development of intimal hyperplasia, which may improve graft patency in the extension of the follow-up of ongoing studies.
La endocarditis infecciosa (EI) es una enfermedad sistémica con afectación multiorgánica. Se trata de una entidad grave, que con frecuencia requiere de tratamiento quirúrgico, a pesar del cual las ...tasas de mortalidad pueden ser elevadas debido a complicaciones derivadas de una respuesta inflamatoria sistémica exacerbada y/o sepsis. Los sistemas de hemadsorción (HA) como Cytosorb® (Cytosorbents Corporation, Nueva Jersey, EE. UU.) permiten la eliminación de citocinas proinflamatorias y endotoxinas para poder mejorar el pronóstico de estos pacientes. Realizamos una búsqueda sistemática en PubMed y Cochrane para identificar trabajos comparativos controlados del uso perioperatorio de Cytosorb® en sujetos intervenidos de cirugía cardiaca por EI. Se efectuó un metaanálisis contrastando diferentes resultados clínicos en el que se incluyeron nueve documentos. La aplicación de la terapia con HA no redujo la mortalidad en el posoperatorio temprano (riesgo relativo RR=0,75; p=0,05). Tanto la duración de la ventilación mecánica (diferencia de medias DM=-6,91 h; p=0,01) como la del soporte vasopresor (DM=-34,47 h; p=0,03) fueron significativamente menores en el caso de los pacientes tratados con HA. Sin embargo, las estancias posoperatorias, la incidencia de fallo renal posoperatorio o la necesidad de revisión quirúrgica por sangrado no presentaron diferencias entre grupos. Como conclusión, la utilización del filtro de HA Cytosorb® en la cirugía cardiaca por EI permitió un mejor control de la actividad inflamatoria posoperatoria, con menores requerimientos de soporte vasoconstrictor y duración de la ventilación mecánica, pero sin impacto sobre la mortalidad perioperatoria ni otras complicaciones relacionadas.
Infective endocarditis (IE) is a systemic disease with multiorgan involvement. It is a severe disease, which frequently requires surgical treatment, despite which mortality rates can be high due to complications related to an uncontrolled systemic inflammatory response and/or sepsis. Hemadsorption systems such as Cytosorb® allow the removal of proinflammatory cytokines and endotoxins to improve the prognosis of these patients. We performed a systematic review in Pubmed and Cochrane to identify controlled comparative studies of the perioperative use of Cytosorb® in patients undergoing cardiac surgery for IE. A meta-analysis was performed comparing different clinical outcomes. 9 studies were finally included in the meta-analysis. The use of hemadsorption therapy did not reduce mortality in the early postoperative period (RR=0.75; p=0.05). Both, the duration of mechanical ventilation (mean difference=-6.91hours; p=0.01) and the duration of support with vasopressor drugs (mean difference=-34.47hours; p=0.03) were significantly shorter in the case of patients treated with hemadsorption. However, postoperative stays, the incidence of postoperative renal failure or the need for surgical revision due to bleeding did not show differences between groups. To conclude, the use of the Cytosorb® hemadsorption filter in cardiac surgery for IE allowed better control of postoperative inflammatory activity, with lower requirements for vasopressor support and duration of mechanical ventilation, but without impact on perioperative mortality or other related complications.
El fibroelastoma papilar es un tumor cardiaco primario benigno localizado normalmente en el endocardio valvular, aunque es posible encontrarlo en otras estructuras endocárdicas. Según estudios ...recientes, podría ser el tumor cardiaco primario benigno más común. En un número importante de casos es asintomático, se diagnostica de manera incidental y, en caso de presentar clínica acompañante, se asocia a eventos cerebrovasculares tromboembólicos. Mostramos nuestra experiencia en el tratamiento quirúrgico del fibroelastoma papilar y se lleva a cabo una revisión bibliográfica de la literatura.
Estudio retrospectivo unicéntrico mediante le revisión de las historias clínicas de todos los pacientes diagnosticados de fibroelastoma papilar y tratados quirúrgicamente en nuestro Servicio.
Desde enero de 2016 hasta octubre de 2022 se intervinieron 14 pacientes, predominantemente varones, con una edad media de 61±12,6 años. El diagnóstico se realizó en casi todos los casos mediante estudios previos a la cirugía. La mayoría de los tumores eran asintomáticos y se localizaron en la válvula aórtica. Ninguno precisó reemplazo o reparación valvular. En 5 casos (35,7%) se realizó crioablación concomitante del lecho tumoral. No hubo mortalidad quirúrgica. Tras 3,2±2,22 años de seguimiento dos pacientes fallecieron de causas no cardiacas. No se han objetivado recidivas.
El tratamiento quirúrgico del fibroelastoma papilar es seguro y ofrece buenos resultados. La crioablación concomitante podría ser útil para conseguir una eliminación tumoral completa sin necesidad de recambio valvular, aunque es necesaria más evidencia. La realización de un registro multicéntrico permitiría un mayor conocimiento de esta patología.
Papillary fibroelastoma is a benign primary cardiac tumor normally located in the valvular endocardium, although it can be found in other endocardial structures. According to recent studies, it could be the most common benign primary cardiac tumor. In a significant number of cases it is asymptomatic, diagnosed incidentally and, in symptomatic cases, usually they present as thromboembolic neurological events. We show our experience in the surgical treatment of papillary fibroelastoma and a review of the literature is performed.
Single-center retrospective study by reviewing the medical records of all patients diagnosed with papillary fibroelastoma and surgically treated in our department.
From January 2016 to October 2022, 14 patients were operated on, predominantly men, with a mean age of 61±12.6 years. The diagnosis was made mostly in almost all cases during the preoperative study for other indications. Most of the tumors were asymptomatic and were located in the aortic valve. No patient required valve replacement or repair. In 5 cases (35.7%) concomitant cryoablation was performed. There was no surgical mortality. After 3.2±2.22 years of follow-up, two patients died of non-cardiac causes. No tumoral recurrences have been found.
Surgical treatment of papillary fibroelastoma is safe and offers good short and mid-term results. Concomitant cryoablation might be useful to achieve complete tumor removal without the need for valve replacement, however more evidence is mandatory. The realization of a multicenter registry would allow a better knowledge of this tumors.
The majority of patients with acute promyelocytic leukemia (APL) manifest a specific chromosomal translocation t(15;17)(q22;q21), characterized by the fusion of RARA and PML genes. However, a ...proportion of APL cases are due to variant translocations, being t(11;17) (q23;q21) the most common amongst them. With the major exception of ZBTB16-RARA t(11;17) APL, these variant APL cases present similar morphological features as classic APL and are characterized by a lack of differentiation response to retinoids.
We describe the case of variant APL with the ZBTB16-RARA fusion gene, showing a distinct morphology of classical APL, characterized by crystalline intracytoplasmic inclusions in both peripheral blood (PB) and bone marrow (BM) patients' blasts. Our patient was treated with two courses of intensive chemotherapy, initiating maintenance treatment with all-trans retinoic acid (ATRA) on day twenty-eight of the second course. Our patient achieved complete remission (CR) once the intensive chemotherapy was combined with ATRA.
This is the second case described of APL with t(11;17) that showed crystalline intracytoplasmic inclusions. The finding of these morphological features may suggest the presence of a variant translocation with RARA, being that both cases described are related to the presence of t(11;17). Despite induction treatment with intensive chemotherapy that included a seven-day continuous treatment with cytarabine (200 mg/m
), plus daily idarubicin (12 mg/m
) during the first three days, our patient did not achieve complete remission (CR) until scheduled 3 + 7 regimen combined with ATRA treatment was established. This observation suggests that ATRA may be partially effective in some ZBTB16-RARA APLs.
El hematoma intramural agudo tipo A (HIM-A) constituye una entidad de elevada letalidad, subsidiaria de intervención quirúrgica emergente. No obstante, casos seleccionados (estabilidad ...clínica/hemodinámica, diámetro<50mm, espesor<11mm) pueden presentar mejor pronóstico, proponiéndose el manejo conservador como alternativa en estudios asiáticos. Por ello, planteamos realizar un metaanálisis comparando la mortalidad hospitalaria del manejo conservador vs. quirúrgico del HIM-A, así como tendencias temporales y diferencias geográficas. Realizamos una búsqueda en Medline® por palabras clave: «aortic intramural hematoma». Se incluyeron estudios comparativos de ambas alternativas terapéuticas con resultados de mortalidad hospitalaria de pacientes con HIM-A. Se valoró el tamaño del efecto, expresado como riesgo relativo (RR), mediante el test de Mantel-Haenszel y un modelo de efectos fijos o aleatorios según el nivel de heterogeneidad I2<o≥25%, respectivamente. Se incluyeron 46 estudios con 890 pacientes para manejo conservador y 1.071 para quirúrgico. Globalmente, el tratamiento quirúrgico fue superior (RR=0,65, p=0,04), y también lo fue en estudios occidentales (RR=0,57, p=0,02). Cuando se excluyeron los estudios que asignaron el tratamiento conservador a enfermos rechazados para cirugía, ambas estrategias terapéuticas fueron equivalentes, globalmente (RR=1,31, p=0,11) y en el medio occidental (RR=1,06, p=0,78), siendo el tratamiento conservador superior en estudios orientales (RR=2,00, p=0,03). El análisis de tendencias temporales mostró una progresiva superioridad del manejo conservador electivo, independientemente del medio considerado. Podemos concluir que, aunque el tratamiento de primera elección del HIM-A en nuestro medio es quirúrgico, determinados grupos de pacientes pueden beneficiarse de una estrategia conservadora como puente a una intervención quirúrgica diferida, e incluso como terapia definitiva.
Type A intramural hematoma (IMH-A) is a highly lethal entity where emergent surgical intervention is usually indicated. However, selected cases (clinical/hemodynamic stability, diameter<50mm, thickness<11mm) may have a better prognosis, and a conservative management as an alternative has been suggested in Asian studies. Thus, we sought to perform a meta-analysis study comparing the hospital mortality of conservative vs. surgical management of HIM-A as well as temporal and geographical trends. We searched studies in Medline® by keywords: “aortic intramural hematoma”. Comparative studies of both therapeutic alternatives that reported hospital mortality of IMH-A patients were included. The effect size, expressed as relative risk (RR), was assessed using the Mantel-Haenszel test using a fixed or random effects model according to the level of heterogeneity I2<or≥25%, respectively. Forty-six studies comprising 890 patients for conservative management and 1,071 for surgery were included. The surgical treatment was superior in the global series (RR=0.65, P=.04), as well as in western studies (RR=0.57, P=.02). When the studies that assigned conservative treatment to patients rejected for surgery were excluded, both therapeutic strategies were equivalent, globally (RR=1.31, P=.11) and in the western environment (RR=1.06, P=.78). Conversely, conservative treatment was superior in oriental studies (RR=2.00, P=.03). Temporal trends analysis showed a progressive superiority of elective conservative management regardless of its geographical origin. We can conclude that, although the surgical treatment of IMH-A is the first treatment choice in our environment, selected groups of patients can benefit from a conservative strategy as a bridge to a delayed surgical intervention or even as definitive therapy.
Abstract Objectives To analyse the effect of the early coadministration of ivabradine and beta-blockers (intervention group) versus beta-blockers alone (control group) in patients hospitalised with ...heart failure and reduced left ventricular ejection fraction (HFrEF). Methods A comparative, randomised study was performed to compare the treatment strategies of beta-blockers alone versus ivabradine and beta-blockers starting 24 hours after hospital admission, for acute HF in patients with an left ventricular ejection fraction (EF) < 40%, sinus rhythm, and a heart rate (HR) > 70 bpm. Results A total of 71 patients were examined, 33 in the intervention group and 38 in the control group. No differences were observed with respect to their baseline characteristics or standard treatment at discharge. HR at 28 days (64.3 ± 7.5 vs. 70.3 ± 9.3 bpm, p = 0.01) and at 4 months (60.6 ± 7.5 vs. 67.8 ± 8 bpm, p = 0.004) after discharge were significantly lower in the intervention group. Significant differences were found with respect to the EF and brain natriuretic peptide levels at 4 months. No differences in clinical events (rehospitalisation/death) were reported at 4 months. No severe side effects attributable to the early administration of ivabradine were observed. Conclusions The early coadministration of ivabradine and beta-blockers during hospital admission for acute HFrEF is feasible and safe, and it produces a significant decrease in HR at 28 days and at 4 months after hospital discharge. It also seemed to improve systolic function and functional and clinical parameters of HF patients at short-term.
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