Generation and External Validation of a Histologic Transformation Risk Model for Patients with Follicular Lymphoma.

Follicular lymphoma (FL) is the most frequent indolent lymphoma. Some patients (10%-15%) experience histologic transformation (HT) to a more aggressive lymphoma, usually diffuse large B-cell lymphoma (DLBCL). This study aimed to validate and improve a genetic risk model to predict HT at diagnosis.We collected mutational data from diagnosis biopsies of 64 FL patients. We combined them with the data from a previously published cohort (total n = 104; 62 from nontransformed and 42 from patients who did transform to DLBCL). This combined cohort was used to develop a nomogram to estimate the risk of HT. Prognostic mutated genes and clinical variables were assessed using Cox regression analysis to generate a risk model. The model was internally validated by bootstrapping and externally validated in an independent cohort. Its performance was evaluated using a concordance index and a calibration curve. The clinicogenetic nomogram included the mutational status of 3 genes (HIST1HE1, KMT2D, and TNFSR14) and high-risk Follicular Lymphoma International Prognostic Index and predicted HT with a concordance index of 0.746. Patients were classified as being at low or high risk of transformation. The probability HT function at 24 months was 0.90 in the low-risk group vs 0.51 in the high-risk group and, at 60 months, 0.71 vs 0.15, respectively. In the external validation cohort, the probability HT function in the low-risk group was 0.86 vs 0.54 in the high-risk group at 24 months, and 0.71 vs 0.32 at 60 months. The concordance index in the external cohort was 0.552. In conclusion, we propose a clinicogenetic risk model to predict FL HT to DLBLC, combining genetic alterations in HIST1H1E, KMT2D, and TNFRSF14 genes and clinical features (Follicular Lymphoma International Prognostic Index) at diagnosis. This model could improve the management of FL patients and allow treatment strategies that would prevent or delay transformation.

18F-FDG-PET/CT response after first-line treatment as a prognostic factor for survival in peripheral T-cell lymphoma: a Spanish retrospective study.

BACKGROUND: An accurate assessment of tumor viability after first-line treatment is critical for predicting treatment failure in peripheral T-cell lymphomas (PTCLs). 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) has been adopted as the preferred assessment method in clinical trials, but its impact in clinical practice should be examined. This study aims to determine the prognostic significance of18F-FDG-PET/CT for survival following first-line treatment in PTCL patients. RESEARCH DESIGN AND METHODS: Retrospective observational study including 175 patients diagnosed with PTCL between 2008 and 2013 in 13 Spanish sites. RESULTS: Fifty patients were evaluated with18F-FDG-PET/CT following first-line therapy: 58% were18F-FDG-PET/CT-negative and 42% were18F-FDG-PET/CT-positive. Disease progression occurred in 37.9% of18F-FDG-PET/CT-negative patients and in 80.9% of18F-FDG-PET/CT-positive patients (p = 0.0037). Median progression-free survival and overall survival were 67 and 74 months for18F-FDG-PET/CT-negative patients, and 5 (p < 0.0001) and 10 months (p < 0.0001), respectively, in18F-FDG-PET/CT-positive patients. After multivariate analysis, only B symptoms emerged as a negative predictive factor of complete response (RR 7.08; 95% CI 1.60-31.31; p = 0.001). CONCLUSIONS: 18F-FDG-PET/CT identifies high-risk PTCL patients who will have poor prognosis and survival following first-line treatment. However, more research is needed to confirm the best treatment options for PTCL patients.

Comparing R-Bendamustine vs. R-CHOP Plus Maintenance Therapy as First-Line Systemic Treatment in Follicular Lymphoma: A Multicenter Retrospective GELTAMO Study.

Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) and R-bendamustine (R-B) are the most common frontline treatment strategies for advanced-stage follicular lymphoma (FL). After R-CHOP induction therapy, using rituximab for maintenance therapy notably improves outcomes; however, whether this can be achieved by using the same approach after R-B therapy is still being determined. This retrospective analysis compared 476 FL patients from 17 GELTAMO centers who received R-based regimens followed by rituximab maintenance therapy for untreated advanced-stage FL. The complete response rate at the end of induction was higher with R-B and relapses were more frequent with R-CHOP. During induction, cytopenias were significantly more frequent with R-CHOP and so was the use of colony-stimulating factors. During maintenance therapy, R-B showed more neutropenia and infectious toxicity. After a median follow-up of 81 months (95% CI: 77-86), the 6-year rates of progression-free survival (PFS) were 79% (95% CI: 72-86) for R-bendamustine vs. 67% (95% CI: 61-73) for R-CHOP (p = 0.046), and 6-year overall survival (OS) values were 91% (95% CI: 86-96) for R-B vs. 91% (95% CI: 87-94) for R-CHOP (p = 0.49). In conclusion, R-B followed by rituximab maintenance therapy in patients with previously untreated FL resulted in significantly longer PFS than R-CHOP, with older patients also benefiting from this treatment without further toxicity. Adverse events during maintenance were more frequent with R-B without impacting mortality.

Rehabilitación cardiorrespiratoria en hipertensión pulmonar: experiencia en un centro de referencia / Cardiorespiratory rehabilitation in pulmonary hypertension: experience in a reference center

RESUMEN Introducción: La hipertensión pulmonar (HP) abarca un grupo heterogéneo de enfermedades que genera discapacidad y aumento de la morbimortalidad. La rehabilitación cardiorrespiratoria (RC) es un recurso terapéutico subutilizado en esta condición. Objetivo: Estimar los efectos de un programa de RC en una prueba de caminata de campo y en la calidad de vida de pacientes con diagnóstico de HP de los grupos I y IV. Materiales y Métodos: Los pacientes fueron evaluados antes y después de la intervención mediante la prueba de caminata de 6 minutos (PC6M) y el Saint George's Respiratory Questionnaire (SGRQ). El programa de RC consistió en 8 semanas de ejercicios supervisados con modalidad institucional. Resultados: Se incluyeron 19 pacientes con diagnóstico de HP precapilar por cateterismo cardíaco derecho, 18 mujeres (94,7%) con una media de edad de 45,5 ± 14,3 años. Trece (68,4%) presentaron HP del grupo I, y 6 (31,6%) HP del grupo IV. Se observaron cambios estadísticamente significativos en la PC6M (diferencia de medias -DM- 31 ± 27,3 metros; p <0,001), y en el SGRQ (DM 8,2 ± 10,2; p<0,01). No se reportaron eventos adversos graves durante el programa. Conclusiones: Nuestro estudio sugiere que un programa de RC supervisado en pacientes con HP podría mejorar la distancia caminada y la calidad de vida.

ABSTRACT Background: Pulmonary hypertension (PH) comprises a heterogeneous group of diseases resulting in disability and increased morbidity and mortality. Cardiopulmonary rehabilitation (CR) is a therapeutic resource not widely used in this condition. Objective: The aim of this study was to evaluate the effects of a CR program on a walking test and on the quality of life in patients with group 1 and group 4 PH Methods: Patients were evaluated before and after the intervention with the six-minute walk test (6MWT) and Saint George's Respiratory Questionnaire (SGRQ). The program consisted of 8 weeks of supervised exercises within the institution. Results: Nineteen patients with precapillary PH diagnosed by right heart catheterization were included; 18 were women (94.7%) with a mean age of 45.5±14.3 years. Thirteen (68.4%) patients had group 1 PH and 6 (31.6%) had group 4 PH. There were statistically significant changes in the 6MWT [mean difference (MD) 31±27.3 m; p<0.001], and in the SGRQ (MD 8.2±10.2; p<0.01). No adverse events were reported during the program. Conclusions: Our study suggests that a supervised CR program in patients with PH could improve the distance walked and the quality of life.