Allogeneic hematopoietic cell transplantation (HCT) is the only curative therapy for myelofibrosis (MF). In this large multicenter retrospective study, overall survival (OS) in MF patients treated with allogeneic HCT (551 patients) and without HCT (non-HCT) (1377 patients) was analyzed with Cox proportional hazards model. Survival analysis stratified by the Dynamic International Prognostic Scoring System (DIPSS) revealed that the first year of treatment arm assignment, due to upfront risk of transplant-related mortality (TRM), HCT was associated with inferior OS compared with non-HCT (non-HCT vs HCT: DIPSS intermediate 1 [Int-1]: hazard ratio [HR] = 0.26, P < .0001; DIPSS-Int-2 and higher: HR, 0.39, P < .0001). Similarly, in the DIPSS low-risk MF group, due to upfront TRM risk, OS was superior with non-HCT therapies compared with HCT in the first-year post treatment arm assignment (HR, 0.16, P = .006). However, after 1 year, OS was not significantly different (HR, 1.38, P = .451). Beyond 1 year of treatment arm assignment, an OS advantage with HCT therapy in Int-1 and higher DIPSS score patients was observed (non-HCT vs HCT: DIPSS-Int-1: HR, 2.64, P < .0001; DIPSS-Int-2 and higher: HR, 2.55, P < .0001). In conclusion, long-term OS advantage with HCT was observed for patients with Int-1 or higher risk MF, but at the cost of early TRM. The magnitude of OS benefit with HCT increased as DIPSS risk score increased and became apparent with longer follow-up.
Myeloproliferative neoplasms (MPNs) are characterized by significant symptom burden. Integrative medicine (IM) offers unique symptom management strategies. This study describes IM interventions utilized by MPN patients and the association with symptom burden, quality of life, depression, and fatigue adjusted for lifestyle confounders. MPN patients were surveyed online for IM utilization, MPN symptom burden (MPN‐Symptom Assessment Form Total Symptom Score), depression (Patient Health Questionnaire), fatigue (Brief Fatigue Inventory), and a single question on overall quality of life. Measures were compared by IM participation and adjusted for alcohol and tobacco use, BMI, diet, and MPN type using multiple linear and logistic regression. A total of 858 participants were included in the analysis. Aerobic activity (p =< 0.001) and strength training (p = 0.01) were associated with lower mean symptom burden while massage (p =< 0.001) and support groups (p =< 0.001) were associated with higher levels of symptom burden. Higher quality of life was reported in massage (p = 0.04) and support groups (p = 0.002) while lower quality of life was noted in aerobic activity (p =< 0.001) and strength training (p = 0.001). A lower depression screening score was noted in those participating in aerobic activity (p = 0.006), yoga (p = 0.03), and strength training (p = 0.02). Lower fatigue was noted in those participating in aerobic activity (p =< 0.001) and strength training (p = 0.03) while higher fatigue was noted in those participating in massage (p =< 0.001) and breathing techniques (p = 0.02). Data available on request from the authors. This international survey of MPN patients on IM usage, has shown that patients who participated in a form of IM had a pattern of decreased levels of symptom burden, fatigue, depression, and higher QoL, as adjusted for health lifestyle practices overall.
Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder with a short survival duration despite the use of novel therapeutics. PCL remains an understudied disease for which there is no current standard of care treatment. Knowledge on optimal novel drug sequencing, including the role and timing of hematopoietic stem cell transplant as primary and salvage therapy is needed. We conducted a large retrospective analysis of 153 PCL patients, using the new definition of ≥ 5% circulating plasma cells, including clinical features and treatment outcomes across seven academic centers in United States. This is, to the best of our knowledge, the largest multicenter study conducted in the US of this rare disease. Disease presentation, clinical and genetic characteristics, and treatment patterns of 93 patients with primary PCL and 57 with secondary PCL were described. Additionally, associations between patient characteristics and mortality were investigated using Cox proportional-hazards regression models. Secondary PCL was associated with a worse prognosis, with a median overall survival of 3.2 months compared to 36.6 months for primary PCL (p
Scleromyxedema is a rare and progressive disease that currently has no standard treatment. Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an effective therapy for scleromyxedema, especially in patients with refractory or relapsed disease.
Background: Myeloproliferative neoplasms (MPNs) are a group of myeloid malignancies associated with significant symptom burden. Despite pharmacological advances in therapies, inadequate management of MPN symptoms results in reduced quality of life. Objective: This study aims to determine the feasibility of a 12-week global wellness mobile app intervention in decreasing MPN symptom burden. The University of Arizona Andrew Weil Center for Integrative Medicine’s global wellness mobile app, My Wellness Coach (MWC), guides patients to improve their health and well-being through facilitating behavior changes. Methods: Of the 30 patients enrolled in a 12-week intervention, 16 (53%) were retained through the final assessment. Feasibility was assessed by the ease of recruitment, participant adherence, and mobile app acceptability. App acceptability was measured using the user version of the Mobile Application Rating Scale. MPN symptom burden was measured at baseline and 12 weeks after the intervention. Results: Recruitment was efficient, with the participant goal reached within a 60-day period, suggestive of a demand for such an intervention. Adherence was less than the target within study design (75%), although similar to mobile device app use in other studies (53%). The app was deemed acceptable based on the mean user version of the Mobile Application Rating Scale 3-star rating by participants. Finally, there were statistically significant improvements in several MPN symptoms, quality of life, and total score on the Myeloproliferative Neoplasm Symptom Assessment Form surveys. Conclusions: Our 12-week intervention with the MWC app was feasible and was associated with a decrease in MPN symptom burden. Further investigation of the MWC app for use as a self-management strategy to reduce the symptom burden in patients with MPN is warranted.
Purpose of Review: Prostate cancer is the second most common type of cancer in men. Its incidence varies widely and is influenced by geographic location, race, ethnicity, lifestyle factors, and diet. The purpose of this review is to discuss the association between prostate cancer and diet and outline the impact of fats, carbohydrates, proteins, vitamins and phytonutrients on the pathogenesis of disease. Recent Findings: Although conclusive evidence is limited, current data is indicative that a diet low in particular fats, animal proteins, dairy products and high in vegetables and fruits can be beneficial in supporting the course of disease. Summary: Promoting a dietary pattern low in processed meat, dairy products, refined carbohydrates and saturated fats, but high in fruits and vegetables may have beneficial effects on prostate metabolism and inhibit various stages of carcinogenesis.