The authors report the case of a 58-year-old female patient with a life-threatening invasive fungal disease caused by a coinfection of Aspergillus and Mucor species that occurred during induction and consolidation chemotherapy for an acute myeloid leukemia. The disease was successfully treated with an aggressive therapeutic approach, which consisted of liposomal amphotericin B in combination with surgical exploration without compromising the treatment of her underlying disease. The case demonstrates the difficulties associated with establishing a diagnosis as well as the need for close observation to identify these infections, which are often misdiagnosed and only suspected late during the course of the disease. Doubts about the reliability of the diagnostic tools lead to uncertainties with regard to the choice of first-line drugs as well the selection of the most appropriate therapeutic strategy in hematologic patients.

Background Multiple myeloma (MM) is the second most common hematological cancer worldwide and has significant morbidity and mortality and is increasing in incidence. While MM management costs are considerable, specific economic data at the country level remain scarce. Objective This study assesses the burden and cost of MM in Portugal from the perspective of the National Health Service (NHS) to support the definition of health policies, resource allocation and patient care. Methods Developed by the Portuguese Multiple Myeloma Group, this study considers the most recent available data. Burden of disease was measured using disability-adjusted life-years (DALYs). The cost of MM was estimated using a prevalence-based model that estimated direct costs for the NHS considering all costs associated with diagnosis, hospitalizations, surgeries, emergency visits, medical appointments, drugs and transportation. Costs were quantified based on the diagnosis-related group funding price, except for drug usage, which was calculated using the average hospital product stock price. Results The burden of disease attributable to MM for 2018 was estimated at 8931 DALYs: 8570 resulting from premature deaths and 361 from disability. Average yearly direct costs per patients with MM amounted to €31,449 (year 2018 values). Total direct costs are estimated at €61 million per year. Conclusions The mortality rate in MM means that most DALYs are due to years of life lost rather than years lost due to disability. This study generates comprehensive data on the burden and cost of MM in Portugal and provides updated insights into the costs associated with the management of MM.

With increasingly effective treatments, early death (ED) has become the dominant reason for therapeutic failure in patients with acute promyelocytic leukemia (APL). To better prevent ED, patients with high-risk of ED must be identified. Our aim was to develop a score that predicts the risk of ED in a real-life setting. We used APL patients in the population-based Swedish AML Registry (n=301) and a Portuguese hospitalbased registry (n=129) as training and validation cohorts, respectively. The cohorts were comparable with respect to age (median 54 and 53 years) and ED rate (19.6% and 18.6%). The score was developed by logistic regression analyses, riskper-quantile assessment and scoring based on ridge regression coefficients from multivariable penalized logistic regression analysis. White blood cell (WBC) count, platelet count and age were selected by this approach as the most significant variables for ED prediction. The score identified low-, high- and very high-risk patients with ED risks of 4.8%, 20.2% and 50.9% respectively in the training cohort and with 6.7%, 25.0% and 36.0% as corresponding values for the validation cohort. The score identified an increased risk of ED already at sub-normal and normal WBC levels and consequently, it was better to predict ED risk than the Sanz score (AUROC 0.77 vs. 0.64). In summary, we here present an externally validated and population-based risk score to predict ED risk in a real-world setting, identifying patients with the most urgent need of aggressive ED prevention. Also, the results suggest increased vigilance for ED already at sub-normal/normal WBC levels.

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Autologous stem cell transplantation (ASCT) is the gold standard therapy for suitable multiple myeloma (MM) patients after induction with high dose therapy. To date, the evidence of a reliable marker of prognosis in these cases remains scarce. Our aim was to evaluate appearance of unrelated atypical serum immunofixation patterns (ASIPs) as a marker of prognosis in MM patients submitted to ASCT. We retrospectively analysed data from 65 patients. Interestingly, we observed that presence of ASIPs was associated with longer progression-free survival and longer overall survival. Our results suggested that presence of ASIPs could be a novel marker of good prognosis in MM patients submitted to ASCT.