Fixed duration vs. prolonged duration treatment after first line therapy in patients with systemic light chain amyloidosis.

BACKGROUND: The main objective of treatment in systemic light chain amyloidosis (AL amyloidosis) is to achieve the best hematological response. Deeper responses are associated with better organ responses and survival. In this study, we analysed the efficacy of prolonged duration treatment after first line in patients with AL amyloidosis. METHODS: Retrospective analysis that included patients older than 18 years with AL amyloidosis. We excluded patients with more than 30% marrow plasmacytosis or concurrent multiple myeloma. Two cohorts identified accordingly if they received or not prolonged treatment after the first line. Survival analysis regarding progression free survival (PFS) and overall survival (OS) estimated with Kaplan-Meier and comparisons between groups with log-rank. RESULTS: Thirty-eight patients were included in the analysis with a median age of 55 years. Twenty-one patients received prolonged duration treatment and 17 did not. In the prolonged duration group, after a median duration of 12 months, the median PFS was 58.8 months. In the fixed duration treatment group, PFS was 30.6 months. The difference was significant with p = .0045 favouring prolonged duration treatment. Organ response was sustained for a longer period in the prolonged duration treatment group. For OS, the difference was not significant. CONCLUSIONS: Prolonged duration treatment in patients with systemic light chain amyloidosis correlated with better PFS and deeper organ responses. Prospective studies are needed to analyse this further.

Is the risk of refeeding syndrome a problem in reaching nutritional requirements? A cohort of patients on enteral nutrition support.

INTRODUCTION: Background: refeeding syndrome (RS) is a metabolic complication associated with nutritional support. The lack of management protocols for patients on nutritional support may lead to the development of RS or undernourishment. Objective: to evaluate adherence to the algorithm for total enteral nutritional support (ASNET), and the efficacy of reaching nutrient requirements in patients at risk of RS. Methods: a cohort study: screening and nutritional evaluation of patients and risk of RS were performed. Adults admitted to hospital for noncritical illness who received exclusively enteral nutrition (EN) were eligible. Patients with RS, intestinal failure or chronic diarrhea were excluded. Adherence to ASNET was evaluated along with the efficacy of nutritional support to reach the optimal protein and energy requirement (OPER) and any associated complications. Patient follow-up ended when the feeding route was changed or upon discharge. Results: a total of 73 patients were included (mean age, 62 ± 16 yrs; BMI, 18.5 ± 4.2 kg/m2), and 55 % were men. All had nutritional risk of developing RS (57, low; 15, high; 1, very high); 34 % had adequate adherence to ASNET, and 33 % managed to cover the total requirement between 4 and 6 days. OPER was reached by 38 % by the fourth day of EN, and adequate adherence to ASNET increased the probability of achieving it (RR, 2.2; 95 % CI, 1.6-3.2, p < 0.0001) without increasing the associated complications. Nonetheless, 36 % developed complications, of whom 96 % did not adhere to ASNET. Conclusion: adherence to ASNET in patients at risk of RS allowed the achievement of OPER safely by day four with fewer associated complications.

INTRODUCCIÓN: Antecedentes: el síndrome de realimentación (SR) es una complicación metabólica asociada al soporte nutricional y la falta de protocolos puede conducir a su desarrollo o a desnutrición. Objetivo: evaluar la adherencia al algoritmo de soporte nutricional enteral total (ASNET) y la eficacia de alcanzar los requisitos de nutrientes en pacientes con riesgo de SR. Métodos: se realizó un estudio de cohortes con evaluación nutricional del paciente y del riesgo de SR. Fueron elegibles los adultos ingresados en el hospital por una enfermedad no crítica que recibían exclusivamente nutrición enteral (NE). Se excluyeron los pacientes con SR, falla intestinal o diarrea crónica. Se evaluaron la adherencia al ASNET y la eficacia para alcanzar el requerimiento óptimo de proteína, energía (OPER) y cualquier complicación asociada. El seguimiento finalizó cuando se cambió la ruta de alimentación o al alta. Resultados: se incluyeron 73 pacientes (edad de 62 ± 16 años, IMC de 18,5 ± 4,2 kg/m2) y el 55 % fueron hombres. Todos tenían riesgo nutricional de desarrollar SR (57 bajo; 15 alto; 1 muy alto). El 34 % presentaron una adherencia adecuada y el 33 % lograron cubrir el requerimiento total entre 4 y 6 días. La OPER se alcanzó en el 38 % al cuarto día de NE, y la adecuada adherencia al ASNET aumentó la probabilidad de lograrla (RR: 2.2; IC 95 %: 1,6-3,2, p < 0,0001) sin incrementar las complicaciones asociadas. No obstante, el 36 % desarrollaron complicaciones y el 96 % de estos casos no se habían adherido al ASNET. Conclusión: la adherencia al ASNET en los pacientes con riesgo de SR permitió lograr la OPER de forma segura al cuarto día y con menos complicaciones asociadas.

Blastic Transformation in Mexican Population With Chronic Myelomonocytic Leukemia.

BACKGROUND: Chronic myelomonocytic leukemia (CMML) is the most aggressive of chronic leukemias, with short overall survival and a high transformation rate to acute leukemia. We investigated the factors related to blastic transformation in a Mexican population treated at a tertiary referral center. PATIENTS AND METHODS: The records of patients with a diagnosis of CMML from 2000 to 2015 were reviewed. A total of 54 patients were included, with a median age of 71 years and an overall survival of 16 months. The patients with incomplete data were excluded. IBM SPSS Statistics, version 21.0, software was used to perform the statistical analysis. RESULTS: The rate of blastic transformation was 33% (18 patients), and the interval time to progression was 9 months (range, 0-87 months). Comparing the patients who did not undergo blastic transformation to those who did, those with progression to acute leukemia tended to be younger (age, 58 vs. 71 years; P = .001), to have a greater peripheral blood blast count (≥ 2% vs. 0%; P = .003), and were more likely to have immature myeloid precursors circulating in the peripheral blood (94% vs. 64%; P = .02). On multivariate analysis, younger age continued to be a statistically significant factor for progression (hazard ratio, 0.97; 95% confidence interval, 0.929-0.987). CONCLUSION: Mexican patients with CMML that progressed to overt acute leukemia were considerably younger, with a higher tumor burden and short overall survival. In this population, it is important to consider more aggressive treatment at diagnosis, focusing on high-dose chemotherapy and hematopoietic stem cell transplantation within a short period.