External validation of the PE-SARD risk score for predicting early bleeding in acute pulmonary embolism in the RIETE Registry.

INTRODUCTION: The PE-SARD score (syncope, anemia, renal dysfunction) was developed to predict the risk of major bleeding in the acute phase of pulmonary embolism (PE). METHODS: We analyzed data from 50,686 patients with acute PE included in the RIETE registry to externally validate the PE-SARD score. We calculated the overall reliability of the PE-SARD score, as well as discrimination and calibration for predicting the risk of major bleeding at 30 days. The performance of PE-SARD was compared to the BACS and PE-CH models. RESULTS: During the first 30 days, 640 patients (1.3 %) had a major bleeding event. The incidence of major bleeding within 30 days was 0.6 % in the PE-SARD-defined low-risk group, 1.5 % in the intermediate-risk group, and 2.5 % in the high-risk group, for an OR of 2.22 (95 % CI, 2.02-2.43) for the intermediate-risk group (vs low-risk group), and 3.94 for the high-risk group (vs low-risk group). The corresponding sensitivity was 81.1 % (intermediate/high vs low risk), and specificity was 85.9 % (95 % CI, 85.8-86.1) (low/intermediate vs high risk). The applicability of PE-SARD was consistent across clinically relevant patient subgroups and over shorter time periods of follow-up (i.e., 3 and 7 days). The C-index was 0.654 and calibration was excellent. The PE-SARD bleeding score improved the major bleeding risk prediction compared with the BACS and PE-CH scores. CONCLUSIONS: The PE-SARD score identifies PE patients with a higher risk of bleeding, which could assist providers for potentially adjusting PE management, in a framework of shared decision-making with individual patients.

PIM1 is a potential therapeutic target for the leukemogenic effects mediated by JAK/STAT pathway mutations in T-ALL/LBL.

Precursor T-cell neoplasms (T-ALL/LBL) are aggressive hematological malignancies that arise from the malignant transformation of immature thymocytes. Despite the JAK/STAT pathway is recurrently altered in these neoplasms, there are not pharmacological inhibitors officially approved for the treatment of T-ALL/LBL patients that present oncogenic JAK/STAT pathway mutations. In the effort to identify potential therapeutic targets for those patients, we followed an alternative approach and focused on their transcriptional profile. We combined the analysis of molecular data from T-ALL/LBL patients with the generation of hematopoietic cellular models to reveal that JAK/STAT pathway mutations are associated with an aberrant transcriptional profile. Specifically, we demonstrate that JAK/STAT pathway mutations induce the overexpression of the PIM1 gene. Moreover, we show that the pan-PIM inhibitor, PIM447, significantly reduces the leukemogenesis, as well as the aberrant activation of c-MYC and mTOR pathways in cells expressing different JAK/STAT pathway mutations, becoming a potential therapeutic opportunity for a relevant subset of T-ALL/LBL patients.

Daratumumab and Nelarabine Treatment as Salvage Therapy for T-Lymphoblastic Lymphoma: A Case Report.

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.

Thrombotic risk and features of patients with inferior vena cava agenesis: a multicentre, retrospective, observational study.

BACKGROUND: Inferior vena cava agenesis (IVCA) is a rare anomaly predisposing affected people to lower-limb venous thrombosis with low frequency of pulmonary embolism. Antenatal thrombosis and inherited thrombophilia have been suggested as causes of IVCA. However, there is little evidence on the clinical course and management of this condition. We designed a patient registry to assess the thrombotic risk and features of IVCA. METHODS: In this this multicentre, retrospective, observational study, we included patients with IVCA diagnosed by routine imaging from 20 hospitals in Spain (n=18), Portugal (n=1), and Italy (n=1). Patients were identified from a systematic search in radiology databases using data extraction software (cohort A) and alternative searches in medical records for confirmed IVCA (cohort B; option allowed when systematic approaches were unapplicable). Primary outcomes were clinical and imaging features, thrombotic risk, phenotype of IVCA-associated thrombosis, anticoagulant treatment, and the results of thrombophilia testing. FINDINGS: We included patients with IVCA diagnosed by routine imaging studies done between Jan 1, 2010, and Dec 31, 2022. In the systematic search, 4 341 333 imaging exams were screened from the radiology databases of eight centres. 122 eligible patients were enrolled in cohort A. A further 95 patients were identified by screening medical records at 12 centres, of whom 88 were eligible and included in cohort B, making a combined cohort of 210 patients. 96 (46%) of 210 patients were female and 200 (95%) were European or Hispanic. 60 (29%) of 210 patients had hepatic IVC interruption, whereas 150 (71%) had extrahepatic IVCA. In cohort A, 65 (53%) of 122 patients had venous thrombosis, with an estimated annual risk of 1·15% (95% CI 0·89-1·46). Extrahepatic IVCA was associated with a greater risk of venous thrombosis than hepatic IVCA (56 [67%] of 84 patients vs nine [24%] of 38 patients, odds ratio 5·31, 95% CI 2·27-12·43; p<0·0001). Analysis of 126 patients with venous thrombosis pooled from cohorts A and B showed early-onset (median age 34·6 years, IQR 23·3-54·3) and recurrent events (50 [40%] of 126 patients). Patients with extrahepatic IVCA had greater proportions of lower-limb venous thrombosis (95 [87%] of 109 vs nine [53%] of 17, p=0·0010) and recurrence (48 [44%] of 109 vs two [12%] of 17, p=0·015), but lower rates of pulmonary embolism (10 [10%] of 99 vs four [33%] of 12, p=0·044) than did patients with hepatic IVCA. 77 (63%) of 122 patients with thrombosis underwent indefinite anticoagulation. 32 (29%) of 111 patients (29 [34%] of 86 with thrombosis) had coexisting thrombophilias. The recurrence risk was lower for patients receiving indefinite anticoagulation (adjusted odds ratio 0·24, 95% CI 0·08-0·61; p=0·010), and greater for thrombophilias (3·19, 1·09-9·32; p=0·034). INTERPRETATION: This evaluation of a large patient cohort demonstrates the high thrombotic burden of IVCA. We have identified two distinct forms of IVCA, hepatic and extrahepatic, suggesting different underlying mechanisms. Beyond clinical characterisation, we draw attention to this orphan disease and highlight the need for its study and improved care. FUNDING: Spanish Society of Thrombosis and Haemostasis, Instituto de Salud Carlos III, FEDER, Fundación Séneca.

Documento de consenso de la Sociedad Española de Patología Digestiva y de la Sociedad Española de Trombosis y Hemostasia sobre hemorragia digestiva masiva no varicosa y anticoagulantes orales de acción directa / Consensus document of the Spanish Society of Digestives Diseases and the Spanish Society of Thrombosis and Haemostasis on massive nonvariceal gastrointestinal bleeding and direct-acting oral anticoagulants

Introducción: la experiencia y el conocimiento de la hemorragia digestiva masiva no varicosa durante el tratamiento con anticoagulantes orales de acción directa son limitados.Objetivos: proporcionar definiciones y recomendaciones basadas en evidencia.Métodos: documento de consenso elaborado por la Sociedad Española de Enfermedades Digestivas y la Sociedad Española de Trombosis y Hemostasia utilizando la metodología Delphi modificada. Se constituyó un panel de 24 gastroenterólogos con experiencia en hemorragia digestiva y se evaluó la construcción de consenso en tres rondas. Las recomendaciones finales se basan en una revisión sistemática de la literatura utilizando el sistema GRADE.Resultados: el acuerdo de los panelistas fue del 91,53 % para los 30 ítems como grupo, porcentaje que mejoró durante las rondas 2 y 3 para los ítems donde la experiencia clínica es menor. El desacuerdo explícito fue sólo del 1,25 %. Se estableció una definición de sangrado gastrointestinal masivo no varicoso en pacientes con anticoagulantes orales de acción directa y se desarrollaron recomendaciones para optimizar el manejo de esta condición.Conclusión: el abordaje de estos pacientes críticos debe ser multidisciplinario y protocolizado, optimizando las decisiones para la identificación temprana del cuadro y la estabilización del paciente de acuerdo con los principios de la reanimación con control de daños. Por tanto, se debe considerar la reversión inmediata de la anticoagulación, preferentemente con antídotos específicos (idarucizumab para dabigatrán y andexanet alfa para inhibidores directos del factor Xa); resucitación hemostática e identificación y manejo de puntos sangrantes. (AU)

Linfoma anaplásico de células grandes asociado a implantes mamarios. Documento de consenso (I): epidemiología, patogenia, clínica y diagnóstico / Breast-implant associated anaplastic large-cell lymphoma. Consensus document (I). Epidemiology, pathogenesis, symptoms and diagnosis

El linfoma anaplásico de células grandes asociado a implantes mamarios (BIA-ALCL según sus siglas en inglés) es un tipo raro de linfoma no Hodgkin que se ha descrito en el contexto de la cirugía reconstructiva y estética de mama mediante implantes. Estos artículos presentan un consenso de la Sociedad Española de Senología y Patología Mamaria (SESPM) con la idea de unificar, en esta primera parte, los criterios de diagnóstico de esta enfermedad describiendo asimismo la epidemiología y la etiopatogenia

Breast implant-associated anaplastic large-cell lymphoma (BIA-ALCL) is a rare type of non-Hodgkin lymphoma that has been described in the context of reconstructive and aesthetic breast implant surgery. These articles present a consensus of the Spanish Society of Senology and Breast Disease (SESPM). In this first part, the aim is to unify the diagnostic criteria of this disease and describe its epidemiology and etiopathogenesis

Linfoma anaplásico de células grandes asociado a implantes mamarios. Documento de consenso parte II: estadificación, tratamiento, pronóstico y seguimiento / Breast implant-associated anaplastic large-cell lymphoma. Consensus document part II: Staging, prognosis and follow-up

El linfoma anaplásico de células grandes asociado a implantes mamarios (BIA-ALCL según sus siglas en inglés) es un tipo raro de linfoma no Hodgkin que se ha descrito en el contexto de la cirugía reconstructiva y estética de mama mediante implantes. Este segundo artículo presenta la parte del consenso de la Sociedad Española de Senología y Patología Mamaria (SESPM) sobre el tratamiento quirúrgico, médico, radioterápico, pronóstico y seguimiento

Breast implant-associated anaplastic large-cell lymphoma (BIA-ALCL) is a rare type of non-Hodgkin lymphoma that has been described in the context of breast implant reconstructive and cosmetic surgery. This second article presents the consensus of the Spanish Society of Senology and Breast Disease (SESPM) on the medical and surgical treatment of this disease, radiotherapy, prognosis and follow-up

Manejo de la fibrilación auricular en los servicios de urgencias hospitalarios: actualización a 2023 del consenso de la Sociedad Española de Medicina de Urgencias (SEMES), la Sociedad Española de Cardiología (SEC) y la Sociedad Española de Trombosis y Hem / Emergency department management of atrial fibrillation: 2023 consensus from the Spanish Society of Emergency Medicine (SEMES), the Spanish Society of Cardiology (SEC), and the Spanish Society of Thrombosis and Hemostasis (SETH)

La fibrilación auricular (FA) es la arritmia sostenida de mayor prevalencia en los servicios de urgencias (SU), y en España presenta una frecuentación elevada y creciente. Esta arritmia es una enfermedad grave, que incrementa la mortalidad y asocia una relevante morbilidad e impacto en la calidad de vida de los pacientes y en el funcionamiento de los servicios sanitarios. La diversidad de aspectos clínicos a considerar y el elevado número de opciones terapéuticas posibles justifican la implementación de estrategias de actuación coordinadas entre los diversos profesionales implicados, con el fin de incrementar la adecuación del tratamiento y optimizar el uso de recursos. Este documento, realizado por un grupo multidisciplinario de expertos en arritmias cardiacas miembros de la Sociedad Española de Medicina de Urgencias y Emergencias, la Sociedad Española de Cardiología y la Sociedad Española de Trombosis y Hemostasia, recoge las recomendaciones para el manejo de la FA en los SU hospitalarios, basadas en la evidencia disponible y adaptadas a las especiales circunstancias de los mismos. En él se analizan con detalle las estrategias de profilaxis tromboembólica, control de frecuencia y control del ritmo, y los aspectos logísticos y diagnósticos relacionados. (AU)

Atrial fibrillation (AF) is the most prevalent sustained arrhythmia managed in emergency departments, and the already high prevalence of this arrhythmia is increasing in Spain. This serious condition associated with increased mortality and morbidity has a negative impact on patient quality of life and the functioning of the health care system. The management of AF requires consideration of diverse clinical variables and a large number of possible therapeutic approaches, justifying action plans to coordinate the work of several medical specialties in the interest of providing appropriate care and optimizing resources. This consensus statement brings together recommendations for emergency department management of AF based on available evidence adapted to special circumstances. The statement was drafted by a multidisciplinary team of specialists from the Spanish Society of Emergency Medicine (SEMES), the Spanish Society of Cardiology (SEC), and the Spanish Society of Thrombosis and Hemostasis (SETH). Strategies for stroke prophylaxis, measures to bring heart rate and heart rhythm under control, and related diagnostic and logistic issues are discussed in detail. (AU)

Tromboembolia pulmonar masiva en una paciente portadora de una inusual combinación de factores genéticos protrombóticos / Massive pulmonary embolism in a patient with an unusual combination of prothrombotic genetic factors

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