Clinical findings in 25 patients with sinonasal or nasopharyngeal extramedullary plasmacytoma in a four-decade single-centre series

Objectives: Extramedullary plasmacytoma in the sinonasal tract or nasopharynx is rare. The aim of the study was to review data on symptoms, clinical findings, treatment and follow-up of plasmacytomas in the sinonasal and nasopharyngeal regions in order to delineate the main clinical characteristics and the optimal management. Method: Twenty-five patients with sinonasal or nasopharyngeal plasmacytoma, diagnosed and treated at the Helsinki University Hospital during a 39-year period from 1975 to 2013 were retrospectively reviewed. Results: There were 18 males and 7 females with a median age of 66 years (range, 36-80). Sixty-eight percent received only radiotherapy or (chemo)radiotherapy. Forty-seven percent of them had a complete response to primary radiotherapy and one patient had a complete response after receiving additional brachytherapy. Four patients were treated primarily with surgery only. Two of them had a local recurrence, but were then successfully treated with radiotherapy. Altogether, four patients received a combination of surgery and (chemo)radiotherapy. Forty-four percent were alive with no evidence of disease after a median follow-up time of 78 months. Forty percent died of their disease and 16% died of other causes. Conclusions: Our study supports radiotherapy as a treatment of choice, but for small tumours surgery alone or in combination with radiotherapy may also be considered. Chinese abstractPeer reviewe

Guidelines for screening and management of late and long-term consequences of myeloma and its treatment

A growing population of long-term survivors of myeloma is now accumulating the ‘late effects’ not only of myeloma itself, but also of several lines of treatment given throughout the course of the disease. It is thus important to recognise the cumulative burden of the disease and treatment-related toxicity in both the stable and active phases of myeloma, some of which is unlikely to be detected by routine monitoring. We summarise here the evidence for the key late effects in long-term survivors of myeloma, including physical and psychosocial consequences (in Parts 1 and 2 respectively), and recommend the use of late-effects screening protocols in detection and intervention. The early recognition of late effects and effective management strategies should lead to an improvement in the management of myeloma patients, although evidence in this area is currently limited and further research is warranted

Real-world studies of patients with multiple myeloma:future perspectives on symptom assessment and therapy

This thesis describes various studies in the field of drug treatment of multiple myeloma, also known as Kahler's disease, and the complications of this condition. The investigations in this thesis were initiated within the Frisian Hemobase research group that focuses on conducting population-wide scientific research in hemato-oncology. This thesis has shown that new treatment options improved the outcomes of some multiple myeloma patients, but that some patients are still in need for more personalized treatment strategies. For example, we have found that the introduction of bortezomib in the treatment of multiple myeloma patients has a beneficial effect on dialysis dependence. On the other hand, the risk of bone fractures in this patient population is unabated high. In addition, nerve damage is a common problem that is underestimated in daily practice, and we have found an association between vitamin D deficiency and nerve damage. We have further investigated the implications of vitamin D for the use in nerve damage by designing a dosing schedule with high doses of vitamin D. Two-thirds of the study population obtained adequate vitamin D after 6 months. A significant decrease in the severity of neuropathy was observed at the end of the study period. Although these results are promising, the study was not designed to confirm the causality between vitamin D levels and PN, and further research should be undertaken to investigate the effect of vitamin D supplementation on the prevalence and severity of neuropathy in newly diagnosed patients with multiple myeloma

Supporting people with active and advanced disease: a rapid review of the evidence

1.1 Background: the NCSI AAD group The National Cancer Survivorship Initiative (NCSI, 2010) was set up in response to the NHS Cancer Reform Strategy (Department of Health, 2007) as a collaboration between Macmillan Cancer Support, the Department of Health and NHS Improvement, with the goal to achieve a better understanding of the experiences of cancer survivors and to advocate for the provision of services to support them. Within this broad remit, it was recognised that there was a particular group of patients whose needs were commonly neglected; people who were experiencing the ongoing effects of cancer beyond first-line treatment but who were not at end of life. The Active and Advanced Disease (AAD) working group was created to consider issues of relevance to such people. 1.2 Aims of this review This project set out to meet the following aim: To review the literature on selected cancers in order to identify implications for the development of services to support patients experiencing difficulties associated with active and advanced disease

The Medical Research Council Myeloma IX trial: the impact on treatment paradigms*

Osteolytic bone disease is a hallmark of symptomatic multiple myeloma. Bisphosphonates have been the mainstay of treatment to preserve skeletal integrity and prevent skeletal-related events in patients with myeloma-related bone disease. Recently, the MRC Myeloma IX trial demonstrated for the first time improved survival and delayed disease progression with the use of an intravenous amino-bisphosphonate, zoledronic acid, vs. an oral agent, clodronate, with intensive and non-intensive anti-myeloma treatment regimens in patients with newly diagnosed multiple myeloma. These results validate a large body of preclinical, translational and other clinical data suggesting anti-myeloma effects of amino-bisphosphonates. In addition, this trial also provided the first head-to-head evidence for superiority of one bisphosphonate over another (zoledronic acid vs. clodronate) for reducing skeletal morbidity in patients with multiple myeloma, as well as a prospective comparison of toxicities. Despite the use of non-bortezomib containing anti-myeloma treatment regimens in the MRC Myeloma IX trial, these results are encouraging and provide an impetus to continue to evaluate current treatment guidelines for myeloma-associated bone disease

Prevalence and prognostic value of monoclonal gammopathy in heart failure patients with preserved ejection fraction: A prospective study

Background: Heart failure (HF) with preserved ejection fraction (HFpEF) and monoclonal gammopathy of uncertain significance (MGUS) are two entities that share pathophysiological mechanisms. The aim herein, was to assess the prevalence of MGUS in patients with HFpEF and no left ventricular (LV) hypertrophy, as well as its association with a pre-specified clinical endpoint at 12 months. Methods: The present study prospectively enrolled 69 patients admitted with HF, with ejection fraction ≥ 50%, and LV wall thickness < 12 mm. All patients were screened for MGUS. Clinical events were determined over a 12 month follow-up. The pre-specified composite clinical endpoint was readmission for heart failure or death. Results: The prevalence of MGUS in this population was 13%. There were no differences in the incidence of the composite clinical endpoint between patients with and without MGUS. Multivariate analysis showed that treatment with angiotensin converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) was associated with fewer clinical events (HR: 0.153, 95% CI: 0.037–0.622, p = 0.009) and indicated a trend to lower risk of readmission for HF and death. Beta-blockers were associated with lower rates of the composite clinical endpoint (HR: 0.192, 95% CI: 0.05–0.736, p = 0.016), readmission for HF (HR: 0.272, 95% CI: 0.087–0.851, p = 0.025) and indicated a trend to lower mortality. Moreover, potassium serum levels > 5 mEq/L were associated with higher rates of the composite endpoint (HR: 6.074, 95% CI: 1.6–22.65,p = 0.007). Conclusions: The prevalence of MGUS in patients with HFpEF without hypertrophy was 3-fold that of the general population. There was no significant correlation between clinical outcomes and the presence of MGUS. Beta-blockers and ACEIs/ARBs reduced the composite of mortality and readmissions for HF in HFpEF patients. Hyperpotassemia was related to worse prognosis