Safety and Efficacy of Propylene Glycol-Free Melphalan in Patients with AL Amyloidosis Undergoing Autologous Stem Cell Transplantation: Results of a Phase II Study.

Patients with systemic light chain (AL) amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation (HDM/SCT) may develop renal and cardiac toxicities potentially exacerbated by the co-solvent propylene glycol in conventional melphalan formulations. We investigated the safety and efficacy of propylene glycol-free melphalan (PGF-Mel) during HDM/SCT in patients with AL amyloidosis (ClinicalTrials.gov identifier NCT02994784). The primary objective of this phase II, open-label study was evaluation for renal dysfunction, new cardiac arrhythmias, and postural hypotension related to autonomic dysfunction. Secondary objectives included time to neutrophil and platelet engraftment, treatment-related mortality (TRM), overall hematologic response, organ response, and number of peritransplantation hospitalizations. Twenty-eight patients with AL amyloidosis enrolled, of whom 27 underwent HDM/SCT. PGF-Mel at 140 to 200 mg/m2 was administered i.v. in 2 equally divided doses. Patients were monitored for up to 30 days after the last administration of PGF-Mel to assess for treatment-related toxicity. Patients were followed for 12 months from the time of treatment with HDM/SCT for evaluation of hematologic and organ responses. Kaplan-Meier analysis was used to estimate progression-free survival. Two patients (7%) developed renal dysfunction, 5 (19%) experienced new cardiac arrhythmias, and 3 (11%) developed orthostatic hypotension. All patients achieved neutrophil and platelet engraftment, at a median of 10 days and 17 days post-HDM/SCT, respectively. TRM on day +100 was 0%. Peritransplantation hospitalization was required for 23 patients (85%). The most common nonhematologic adverse events were diarrhea (93%), fatigue (82%), and nausea (74%). At 6 months post-HDM/SCT, hematologic complete response or very good partial response occurred in 66% of the patients. At 12 months post-HDM/SCT, renal response occurred in 12 of 23 (52%) patients with renal involvement, and cardiac response occurred in 3 of 11 (27%) patients with evaluable cardiac involvement. Our data indicate that PGF-Mel is safe and efficacious as a high-dose conditioning regimen for autologous SCT in patients with AL amyloidosis.

A challenging case of granulomatosis with polyangiitis with cardiac involvement: a rare case report.

Background: Granulomatosis with polyangiitis (GPA), a systemic antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, is characterized by inflammation of the small arteries, arterioles, and capillaries classically manifesting with glomerulonephritis and necrotizing granulomatous lesions of the upper and lower respiratory tract. With an incidence of approximately 12 cases per one million individuals per year it is an uncommon diagnosis that typically presents as frequent pulmonary and sinus infections; however, if left without definitive treatment progresses to more severe manifestations specifically hemoptysis and hematuria. Case Description: This case report highlights a 15-year-old woman who had both classic and non-classic findings making the diagnosis challenging. Specifically, her age of presentation, improvement with anti-microbials, and coronary dilation were not classic. Additionally, her lab work was negative for the cytoplasmic subset antineutrophil cytoplasmic autoantibody (c-ANCA), but positive for serum anti-proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA) which further delayed the ultimate diagnosis as this is typically c-ANCA positive. Conclusions: Other systemic vasculitides, such as mucocutaneous lymph node disease, are associated with cardiac pathology necessitating further medical management and follow-up to prevent increased morbidity and mortality. Knowing this, we conclude that further evaluation for cardiac pathology would be prudent as part of the initial workup of patients with a diagnosis of GPA. Included is a brief review of available literature on GPA to emphasize the typical presentation, lab findings, and importance of early diagnosis.

Usability Evaluation of a Noninvasive Neutropenia Screening Device (PointCheck) for Patients Undergoing Cancer Chemotherapy: Mixed Methods Observational Study.

BACKGROUND: Patients with cancer undergoing cytotoxic chemotherapy face an elevated risk of developing serious infection as a consequence of their treatment, which lowers their white blood cell count and, more specifically, their absolute neutrophil count. This condition is known as neutropenia. Neutropenia accompanied by a fever is referred to as febrile neutropenia, a common side effect of chemotherapy with a high mortality rate. The timely detection of severe neutropenia (<500 absolute neutrophil count/µL) is critical in detecting and managing febrile neutropenia. Current methods rely on blood draws, which limit them to clinical settings and do not allow frequent or portable monitoring. In this study, we demonstrated the usability of PointCheck, a noninvasive device for neutropenia screening, in a simulated home environment without clinical supervision. PointCheck automatically performs microscopy through the skin of the finger to image the blood flowing through superficial microcapillaries and enables the remote monitoring of neutropenia status, without requiring venipuncture. OBJECTIVE: This study aimed to evaluate the usability of PointCheck, a noninvasive optical technology for screening severe neutropenia, with the goal of identifying potential user interface, functionality, and design issues from the perspective of untrained users. METHODS: We conducted a multicenter study using quantitative and qualitative approaches to evaluate the usability of PointCheck across 154 untrained participants. We used a mixed method approach to gather usability data through user testing observations, a short-answer qualitative questionnaire, and a standardized quantitative System Usability Scale (SUS) survey to assess perceived usability and satisfaction. RESULTS: Of the 154 participants, we found that 108 (70.1%) scored above 80.8 on the SUS across all sites, with a mean SUS score of 86.1 across all sites. Furthermore, the SUS results indicated that, out of the 151 users who completed the SUS survey, 145 (96%) found that they learned how to use PointCheck very quickly, and 141 (93.4%) felt very confident when using the device. CONCLUSIONS: We have shown that PointCheck, a novel technology for noninvasive, home-based neutropenia detection, can be safely and effectively operated by first-time users. In a simulated home environment, these users found it easy to use, with a mean SUS score of 86.1, indicating an excellent perception of usability and placing this device within the top tenth percentile of systems evaluated for usability by the SUS. TRIAL REGISTRATION: ClinicalTrials.gov NCT04448314; https://clinicaltrials.gov/ct2/show/NCT04448314 (Hospital Universitario 12 de Octubre registration) and NCT04448301; https://clinicaltrials.gov/ct2/show/NCT04448301 (Boston Medical Center registration).

Understanding Amyloidosis: Unraveling the Complexities and Therapeutic Approaches for Oncology Nurses.

BACKGROUND:  Primary systemic light-chain (AL) amyloidosis is a rare clonal plasma cell disorder characterized by the production of abnormal immunoglobulin fragments, which form insoluble fibrils that aggregate as amyloid deposits in organs and tissues, leading to organ dysfunction and death. OBJECTIVES:  The aim of this literature review is to increase awareness of AL amyloidosis and educate nurses on the care of this patient population. METHODS:  This overview is based on a literature search of AL amyloidosis, including its pathogenesis, prognosis, and presentation. Guidance for nursing assessment, intervention, and patient education throughout the disease trajectory is presented. FINDINGS:  AL amyloidosis is a rare disease resulting in organ impairment and death if untreated. Nursing management includes knowledge of key assessment, monitoring, intervention, and education strategies with goals to preserve organ function and improve survival and quality of life in patients with AL amyloidosis.

Use of safflower as a trap crop for managing the mirid bug, Lygus pratensis Linnaeus (Hemiptera: Miridae), in cotton fields.

BACKGROUND: Cotton has been increasingly harmed by the mirid bug (Lygus pratensis Linnaeus) in Xinjiang Uyghur Autonomous Region, China. Using trap plants within or around the border of the cotton may be a beneficial management strategy for this pest of cotton. RESULTS: The potential of safflower (Carthamus tinctorius Linn) as a trap plant for managing L. pratensis was evaluated in laboratory and field experiments. Y-tube olfactometer assays demonstrated that L. pratensis was highly attracted to volatiles derived from safflower. Field experiments showed that safflower plots hosted more L. pratensis (adults and nymphs) than cotton plots. Early-sown safflower had more L. pratensis than mid-sown or late-sown safflower, and was more conducive to the settlement and reproduction of L. pratensis. The density of L. pratensis on safflower trap crops in three sowing patterns was significantly higher than on adjacent cotton. The pattern of intercropping safflower trap crops was more effective at reducing densities of L. pratensis on cotton than placing safflower as 'spot' trap crops or peripheral trap crops. However, this result also may be related to the overall area of the safflower trap crops. With regular chemical control of L. pratensis on safflower trap crops, the number of cotton bolls was increased by 10.04%, whereas the rate of boll damage was reduced by 33.44%, compared to cotton without safflower trap crops and insecticide sprays. CONCLUSION: Safflower shows promise as an effective trap crop for L. pratensis, and may contribute to controlling L. pratensis in cotton. © 2020 Society of Chemical Industry.

Impact of Bt Brinjal Cultivation in the Market Value Chain in Five Districts of Bangladesh.

Eggplant (brinjal) is a popular vegetable that provides an important source of income for small, resource-poor Bangladeshi farmers. The biggest constraint to brinjal production is the eggplant fruit and shoot borer (EFSB). This study was conducted in 2019 in five districts in Bangladesh and examined the impacts of using genetically engineered, insect-resistant brinjal (Bt brinjal) on its value and marketing. Based on a survey of Bt and non-Bt farmers, results indicate that Bt brinjal provided an average of 19.6% higher yield and 21.7% higher revenue compared to non-Bt varieties. On a per tonne basis, the revenue benefit of using Bt brinjal was 1.7%, reflecting different levels of acceptability among trade buyers and consumers. Some were prepared to pay higher prices for Bt brinjal compared to non-Bt brinjal because the fruit was less damaged, while others paid a price discount because the Bt brinjal was not available in preferred local varieties. Labor use, expressed in 8-h days, for harvesting, grading, and packaging of Bt brinjal was 14% higher for Bt brinjal, reflecting the increased yields of Bt brinjal. 83.1% of Bt brinjal growers were satisfied with the yields obtained, and 80.6% were satisfied with the quality of fruit. This contrasts with non-Bt brinjal growers where 58.7% were satisfied with their yields and 28% indicated that a large portion of their fruit was infested, not a concern for Bt brinjal. Three-quarters of Bt brinjal growers planned to plant Bt brinjal next season because of the apparent benefits achieved of higher yields, revenue and fruit quality. Many also highlighted the benefits of reduced insecticides. Of the non-Bt growers, 39.6% had not heard of Bt brinjal. However, after hearing more about the impact of the technology, 71.4% of them indicated they planned to grow Bt brinjal next season. These findings suggest there are significant benefits of Bt brinjal and highlight the importance of making the technology available in more varieties that are suitable to local conditions and consumer preferences. Additional studies are warranted to corroborate these findings and explore in more detail the factors influencing decisions made by farmers and consumers regarding Bt brinjal.

Patient outcomes in light chain (AL) amyloidosis: The clock is ticking from symptoms to diagnosis.

INTRODUCTION: Symptomology of AL amyloidosis can be vague, with a broad range of manifestations and potential etiologies. We sought to determine whether time from initial patient-reported symptom onset to diagnosis was associated with survival. METHODS: The Boston University Amyloidosis Patient Database was queried for patients with AL amyloidosis who presented to the Center for initial evaluation from 2010 to 2015. RESULTS: A total of 324 patients with AL amyloidosis were evaluated for initial evaluation. The median time to diagnosis from initial symptom onset was 7.1 months (range, 0-61). At data cutoff, 60.2% (n = 195) of patients were alive; of those, the majority were diagnosed <6 months from initial symptoms (52.3%, n = 102). In contrast, time to diagnosis from symptom onset was >6 months in 63.6% (n = 82) of patients who did not survive at the time of data cutoff (P = .0005). Survival analysis of time from diagnosis to death or data cutoff stratified by time from patient-reported symptom onset to diagnosis (<6, 6-12, and >12 months) showed significant differences among groups (P = .001). Additionally, multivariable regression demonstrated that an increase in time from self-reported symptom onset to diagnosis was significantly associated with an increased risk of death (HR = 1.02, 95% CI = 1.01-1.04, P = .002). CONCLUSION: These results support the importance of early diagnosis for patients with AL amyloidosis.

Safety, tolerability, and response rates of daratumumab in relapsed AL amyloidosis: results of a phase 2 study.

Daratumumab, a monoclonal CD38 antibody, is approved in the treatment of myeloma, but its efficacy and safety in light-chain (AL) amyloidosis has not been formally studied. This prospective phase 2 trial of daratumumab monotherapy for the treatment of AL amyloidosis was designed to determine the safety, tolerability, and hematologic and clinical response. Daratumumab 16 mg/kg was administered by IV infusion once weekly for weeks 1 to 8, every 2 weeks for weeks 9 to 24, and every 4 weeks thereafter until progression or unacceptable toxicity, for up to 24 months. Twenty-two patients with previously treated AL amyloidosis were enrolled. The majority of the patients had received high-dose melphalan and stem cell transplantation and/or treatment with a proteasome inhibitor. The median time between prior therapy and trial enrollment was 9 months (range, 1-180 months). No grade 3-4 infusion-related reactions occurred. The most common grade ≥3 adverse events included respiratory infections (n = 4; 18%) and atrial fibrillation (n = 4, 18%). Hematologic complete and very-good-partial response occurred in 86% of patients. The median time to first and best hematologic response was 4 weeks and 3 months, respectively. Renal response occurred in 10 of 15 patients (67%) with renal involvement and cardiac response occurred in 7 of 14 patients (50%) with cardiac involvement. In summary, daratumumab is well tolerated in patients with relapsed AL amyloidosis and leads to rapid and deep hematologic responses and organ responses. This trial was registered at www.clinicaltrials.gov as #NCT02841033.

A flavin-dependent monooxgenase confers resistance to chlorantraniliprole in the diamondback moth, Plutella xylostella.

The diamondback moth, Plutella xylostella, is a damaging pest of cruciferous crops, and has evolved resistance to many of the insecticides used for control, including members of the diamide class. Previous work on the molecular basis of resistance to diamides has documented mutations in the target-site, the ryanodine receptor, in resistant populations of P. xylostella worldwide. In contrast the role of metabolic resistance to this insecticide class is significantly less clear. Here we show that overexpression of a flavin-dependent monooxgenase (FMO) confers resistance to the diamide chlorantraniliprole in P. xylostella. Transcriptome profiling of diamide resistant strains, with and without target-site resistance, revealed constitutive over-expression of several transcripts encoding detoxification enzymes compared to susceptible strains. Two of these, CYP6BG1, and PxFMO2 were particularly highly overexpressed (33,000 and 14,700-fold, respectively) in a resistant strain (HAW) lacking target-site resistance. After 17 generations without diamide selection the resistance of the HAW strain fell by 52-fold and the expression of PxFMO2 by > 1300-fold, however, the expression of CYP6BG1 declined by only 3-fold. Generation of transgenic Drosophila melanogaster expressing these genes demonstrated that PxFMO2, but not CYP6BG1, confers resistance in vivo. Overexpression of PxFMO2 in the HAW strain is associated with mutations, including a putative transposable element insertion, in the promoter of this gene. These enhance the expression of a reporter gene when expressed in a lepidopteran cell line suggesting they are, at least in part, responsible for the overexpression of PxFMO2 in the resistant strain. Our results provide new evidence that insect FMOs can be recruited to provide resistance to synthetic insecticides.

Odors from phylogenetically-distant plants to Brassicaceae repel an herbivorous Brassica specialist.

Specialist insect herbivores are constrained by highly specific odor recognition systems to accept suitable host plants. Given that odor recognition leads specialist insects to accept a limited range of plants, we hypothesized that phylogenetically distant plants produce odors that are physicochemically different from host odors and would be less attractive or even repellent to a specialist herbivore. We tested this hypothesis by examining behavioral and ovipositional responses of swede midge (Contarinia nasturtii, Diptera: Cecidomyiidae), a specialist of brassicas, to broccoli sprayed with non-host essential oils. Specifically, we asked: (1) How do essential oils from different plant species influence host-seeking and oviposition behaviors of swede midge? (2) Do odors from non-host plants that are not phylogenetically related or physicochemically similar to host plants affect host-seeking or ovipositional behavior of swede midge? In oviposition assays, we found that non-host odors varied in their ability to modify female midge behavior and that phylogenetic relatedness was negatively correlated with larval density. In y-tube assays, we found that female midges most frequently avoided non-host odors that were more similar to brassica odors. Females were less likely to oviposit on or choose any treated host plants, but particularly avoided garlic, spearmint, thyme, eucalyptus lemon, and cinnamon bark treatments. Overall, we found that plant phylogenetic relatedness and odor similarity are related to repellency. Therefore, altering the diversity of plant odors by explicitly accounting for plant phylogenetic distance and odor similarity, relative to host plants, may be an important, underexploited tactic for sustainably managing challenging pests.

Susceptibility of Field Populations of Eggplant Fruit and Shoot Borer (Leucinodes orbonalis Guenée) to Cry1Ac, the Protein Expressed in Bt Eggplant (Solanum melongena L.) in Bangladesh.

Eggplant (Solanum melongena Linn.), or brinjal, was engineered to express an insecticidal protein (Cry1Ac) from Bacillus thuringiensis (Bt) and commercialized in Bangladesh on a limited basis in 2014. As part of an insect resistance management strategy, studies were conducted to determine the susceptibility of the targeted insect pest, the eggplant fruit and shoot borer, Leucinodes orbonalis (Guenée), to Cry1Ac using a diet-incorporation bioassay method. Eighteen populations of L. orbonalis were collected from the main brinjal growing areas in 17 districts of Bangladesh during 2018-2019 and assayed. Larvae from each population were reared to adults and allowed to mate. Eggs from the matings were allowed to hatch, and neonates were used for bioassays. Bioassays were performed with different concentrations of Cry1Ac and an untreated control. Median lethal concentrations (LC50) ranged between 0.035 and 0.358 ppm and molt inhibitory concentration (MIC50) values ranged from 0.008 to 0.181 ppm. Variation in susceptibility among field populations was 10.22-fold for LC50 and 22.63-fold for MIC50. These results were compared to values from 73 populations in India. Overall, the results showed similar natural variation and suggest that these Bangladeshi values can be used as benchmarks for resistance monitoring as Bt brinjal becomes more widely adopted in Bangladesh.

Screening Sugars Can Benefit the Parasitoid Cotesia chilonis (Hymenoptera: Braconidae) Without Benefiting Its Host, Chilo suppressalis (Lepidoptera: Crambidae).

Most adult parasitoids depend on carbohydrate-rich food as an energy source for longevity, fecundity, and mobility. Thus, providing sugars has been proposed as a technique to maximize the biological control efficacy of parasitoids. However, the sugars provided for parasitoids need to be carefully selected because herbivore hosts might also benefit. Here we explore the effects of 12 naturally occurring sugars on the longevity and fecundity of the rice pest, Chilo suppressalis, and the longevity of its parasitoid, Cotesia chilonis, as well as the effect of sugars on sugar consumption of C. chilonis. Results showed that none of the tested sugars significantly impacted the longevity of C. suppressalis, but fructose, glucose, maltose, and sucrose significantly increased its fecundity. The longevity of C. chilonis was significantly increased when fed fructose, glucose, sucrose, maltose, trehalose, and melezitose. Our data suggest that C. chilonis consumed larger quantities of glucose, fructose, and sucrose followed by maltose, melezitose, and trehalose and the longevity of C. chilonis was positively correlated to sugar consumption. Our data also suggest that the herbivore C. suppressalis and its parasitoid C. chilonis responded differently to trehalose and melezitose. Although additional studies are needed, our data suggest that these sugars could be considered as candidate components for sugar sprays to enhance the activity and efficacy of C. chilonis, but without benefiting its pest host.

Bt Brinjal in Bangladesh: The First Genetically Engineered Food Crop in a Developing Country.

Eggplant, or brinjal (Solanum melongena), is a popularly consumed vegetable grown throughout Asia that is prone to vicious and sustained attack by the eggplant fruit and shoot borer (EFSB) (Leucinodes orbonalis) throughout the growing season. Yield losses in Bangladesh because of EFSB infestation have been reported as high as 86%. Farmers reduce crop losses by frequent applications of insecticide. To counter the EFSB pest, Bangladesh has developed and released four Bt brinjal varieties expressing Cry1Ac (Bt brinjal). Bangladesh is the first developing country to release a commercial genetically engineered (GE) food crop. In this article, we discuss the development and adoption of Bt brinjal in Bangladesh from initial distribution to 20 farmers in 2014 to cultivation by more than 27,000 farmers in 2018. Bt brinjal provides essentially complete control of EFSB, dramatically reduces insecticide sprays, provides a sixfold increase in grower profit, and does not affect nontarget arthropod biodiversity. A major focus is to ensure its durability through stewardship. Bangladesh has shown great leadership in adopting biotechnology for the benefit of its farmers and serves as an example for other countries.

Response to a Synthetic Pheromone Source by OX4319L, a Self-Limiting Diamondback Moth (Lepidoptera: Plutellidae) Strain, and Field Dispersal Characteristics of its Progenitor Strain.

The diamondback moth, Plutella xylostella L. (Lepidoptera: Plutellidae), is a global pest that infests vegetable and field crops within the Brassica family. A genetically engineered strain of P. xylostella, OX4319L, carrying a 'self-limiting' gene, has shown potential for managing P. xylostella populations, using sustained releases of OX4319L male moths. In order for such a strain to provide control, the transgenic individuals must exhibit attraction to female P. xylostella sex pheromone and adequate dispersal in the field. In this study, we tested these key traits. First, we compared the responses of the OX4319L male moths to a synthetic female sex pheromone source in wind tunnel trials to those of males from three other strains. We found that OX4319L males responded comparably to strains of non-engineered males, with all males flying upwind towards the pheromone source. Second, we used mark-release-recapture studies of a wildtype P. xylostella strain, from which the OX4319L strain was originally developed, to assess dispersal under field conditions. Released males were recaptured using both pheromone-baited and passive traps within a 2.83 ha circular cabbage field, with a recapture rate of 7.93%. Males were recaptured up to the boundary of the field at 95 m from the central release point. The median dispersal of males was 14 m. These results showed the progenitor strain of OX4319L retained its ability to disperse within a host field. The results of these experiments are discussed in relation to the potential for the effective use of engineered male-selecting P. xylostella strains under field conditions.

Genetically Engineered Crops: Importance of Diversified Integrated Pest Management for Agricultural Sustainability.

As the global population continues to expand, utilizing an integrated approach to pest management will be critically important for food security, agricultural sustainability, and environmental protection. Genetically engineered (GE) crops that provide protection against insects and diseases, or tolerance to herbicides are important tools that complement a diversified integrated pest management (IPM) plan. However, despite the advantages that GE crops may bring for simplifying the approach and improving efficiency of pest and weed control, there are also challenges for successful implementation and sustainable use. This paper considers how several GE traits, including those that confer protection against insects by expression of proteins from Bacillus thuringiensis (Bt), traits that confer tolerance to herbicides, and RNAi-based traits that confer resistance to viral pathogens, can be key elements of a diversified IPM plan for several different crops in both developed and developing countries. Additionally, we highlight the importance of community engagement and extension, strong partnership between industry, regulators and farmers, and education and training programs, for achieving long-term success. By leveraging the experiences gained with these GE crops, understanding the limitations of the technology, and considering the successes and failures of GE traits in IPM plans for different crops and regions, we can improve the sustainability and versatility of IPM plans that incorporate these and future technologies.

Assessment of Yersinia entomophaga as a control agent of the diamondback moth Plutella xylostella.

The application of the biocontrol bacterium Yersinia entomophaga as a foliar spray was assessed for its efficacy against larvae of the diamondback moth, Plutella xylostella. The bacterium was applied as either a broth suspension, or as a biopolymer-based gel foliar spray and compared with commercial insecticides Dipel (Bacillus thuringiensis) and Spinosad. The performance of Y. entomophaga was comparable with that of Dipel. The gel-based formulation extended leaf persistence over that of the basic broth culture spray, while also providing higher initial foliar deposition rates. The bacterium was found to multiply within the P. xylostella larvae to 5.8 × 105 cells per larva, while the median lethal dose (LD50) was determined to be 2.69 × 103 cells per larva. Importantly, B. thuringiensis Cry1A-resistant, Cry1C-resistant, indoxacarb/pyrethroid-resistant, and Spinosad-resistant P. xylostella larvae were susceptible to Y. entomophaga.

Induction Therapy with Bortezomib and Dexamethasone and Conditioning with High-Dose Melphalan and Bortezomib Followed by Autologous Stem Cell Transplantation for Immunoglobulin Light Chain Amyloidosis: Long-Term Follow-Up Analysis.

In immunoglobulin light-chain (AL) amyloidosis, the depth of hematologic response to treatment is associated with improved survival and organ responses. We conducted a clinical trial using bortezomib in induction and in conditioning with melphalan before stem cell transplantation (SCT) for AL amyloidosis. The results of this clinical trial with a median follow-up of 36 months have been reported previously. Here we report the long-term results of this clinical trial with a median follow-up of 77 months. We describe survival, durability of hematologic and organ responses, and relapse rates. Thirty-five patients were enrolled between 2010 and 2013. Hematologic complete response and very good partial response (VGPR) were noted in 100% (27 of 27) of the evaluable patients at 6 months post-SCT. Four patients (15%) had hematologic relapse at a median of 42 months, and 1 patient (3.7%) had organ progression despite maintaining a VGPR at 37 months. The median overall survival and progression-free survival have not yet been reached at the time of this report. Renal and cardiac responses occurred in 65% and 88%, respectively, at 5 years post-SCT. The median time to renal and cardiac response was 12 months and 6 months, respectively. In conclusion, incorporating bortezomib into induction and conditioning yielded durable hematologic responses of AL amyloidosis, with corresponding organ responses and prolonged survival.