We retrospectively analyzed 51 patients with solitary plasmacytoma diagnosed from October 2002 to September 2018 from a cohort of 3575 patients with plasma cell dyscrasias registered in the Kansai ...Myeloma Forum. Twenty-seven patients had solitary bone plasmacytoma (SBP) and 24 had extramedullary plasmacytoma (EMP), with prevalence of 0.8% and 0.7%, respectively. The most frequent M protein was IgG (40%) in SBP, whereas non-secretory proteins were most frequent (50%) in EMP. Five-year overall survival was 78.2% in SBP and 80.8% in EMP (
P
= 0.894). Among patients with SBP, 44% progressed to MM with a median time of 10.5 months (2.4–93.3 months), whereas 8% of EMP patients progressed to MM with a median time of 18.6 months (13.0–24.2 months). The most frequent treatment was radiotherapy (41%) or observation (41%) in SBP, and chemotherapy (54%) in EMP. No statistically significant difference was observed upon univariate analysis of prognostic factors including age, sex, performance status, and IgG M protein. Our results suggest that there are biological differences between SBP and EMP in real-world settings.
Hereditary motor sensory neuropathy type VI (HMSN VI) is hereditary neuropathy accompanied by optic neuropathy. The feasibility of Coenzyme Q10 (CoQ10) as a treatment for subacute visual impairment ...of HMSN VI was examined. A 37-year-old patient with HMSN VI with a novel mitofusin 2 mutation was treated with high dose of CoQ10 (200 mg/day) for eight months. Visual impairment was partially resolved after CoQ10 therapy. High dose CoQ10 therapy may improve the prognosis of subacute visual impairment in HMSN VI. To confirm the effectiveness of CoQ10 on HMSN VI, further studies are needed.
Background
Combinatory strategies with carfilzomib (CFZ), a second‐generation proteasome inhibitor, plus dexamethasone (DEX) with or without lenalidomide (LEN) have shown promising efficacy for ...patients with relapsed/refractory multiple myeloma (RRMM) in pivotal clinical trials. However, their effects on patients who were resistance to bortezomib (BTZ) and/or LEN have not been fully evaluated in a daily practice setting.
Aims
To evaluate the real‐world efficacy and safety of CFZ‐based treatments; that is, CFZ with LEN plus DEX (KRD therapy) and CFZ with DEX (KD therapy), in Asian patients, we conducted a multicenter pilot prospective observational study in the Kyoto Clinical Hematology Study Group.
Methods and Results
All 50 patients with RRMM enrolled in this study were treated with CFZ‐based treatments between 2017 and 2019. KRD and KD were administered to 31 and 19 patients, respectively. The overall response rates (ORRs) were 80.6% with KRD and 73.7% with KD. Two‐year progression‐free survival (PFS) and overall survival (OS) were 58.5% and 79.7% with KRD, and 23.1% and 52.6% with KD. By multivariate analysis, refractoriness to BTZ and to LEN were identified as independent unfavorable factors for both PFS and OS. The common non‐hematologic AEs included hypertension (42.0%), fever (24.0%), fatigue (24.0%), and infection (16.0%). No serious heart failure was observed. This study is registered as UMIN000025108.
Conclusion
This study suggests the need of the development of novel CFZ‐containing strategy which can overcome the refractoriness to BTZ and/or LEN, while both KRD and KD were shown to be mostly feasible in Asian patients in a daily practice setting.
Purpose Continuous electrical stimulation of abdominal wall muscles is known to induce mild muscle fatigue. However, it is not clear whether this is also true for functional electrical stimulation ...delivered only during the expiratory phase of breathing. This study aimed to examine whether or not intermittent electrical stimulation delivered to abdominal wall muscles induces muscle fatigue. Subjects and Methods The subjects were nine healthy adults. Abdominal electrical stimulation was applied for 1.5 seconds from the start of expiration and then turned off during inspiration. The electrodes were attached to both sides of the abdomen at the lower margin of the 12th rib. Abdominal electrical stimulation was delivered for 15 minutes with the subject in a seated position. Expiratory flow was measured during stimulus. Trunk flexor torque and electromyography activity were measured to evaluate abdominal muscle fatigue. Results The mean stimulation on/off ratio was 1:2.3. The declining rate of abdominal muscle torque was 61.1 ± 19.1% before stimulus and 56.5 ± 20.9% after stimulus, not significantly different. The declining rate of mean power frequency was 47.8 ± 11.7% before stimulus and 47.9 ± 10.2% after stimulus, not significantly different. Conclusion It was found that intermittent electrical stimulation to abdominal muscles synchronized with the expiratory would not induce muscle fatigue.
We analyzed the complete mitochondrial genome of the invasive Asian hornet Vespa velutina from Kyushu Island, Japan. The mitochondrial genome of V. velutina was identified as a circular molecule of ...16,388 bp. We predicted that the genome contains 13 protein-coding genes (PCGs), 22 tRNA genes, and 2 rRNA genes, along with one A + T-rich control region. The average AT content is 81.68%. Molecular phylogenetic analysis using the 13 mitochondrial PCGs from 11 closely related taxa of Vespidae indicated that the V. velutina invading the Japanese Islands of Kyushu and Tsushima have a common origin.
To evaluate the specific prognostic value of CAs, we conducted an analysis of 923 symptomatic multiple myeloma patients. Among this cohort, 480 patients had complete data set of high-risk CAs by ...interphase fluorescent in situ hybridization at diagnosis. In the high-risk group analysis, the median OS of patients without CAs (
= 338, 72 %) was 6.5 years, patients with del(17p) (
= 42, 9 %) was 4.4 years, patients with t(4;14) or t(14;16) (
= 72, 15 %) was 4.4 years, and patients with double-positive CAs(del(17p) and t(4;14) or t(14;16)) (
= 18, 4 %) was 2.1 years (
= 0.032). Patients with double-positive CAs had a significantly worse prognosis.
Background: Daratumumab is one of the most widely used treatments for relapsed/refractory multiple myeloma (MM) patients. However, not all patients achieve a lasting therapeutic response with ...daratumumab. Objectives: We hypothesized that a durable response to daratumumab could be predicted by the balance between the MM tumor burden and host immune status. Design: We conducted a retrospective study using the real-world data in the Kansai Myeloma Forum (KMF) database. Methods: We retrospectively analyzed 324 relapsed/refractory MM patients who were treated with daratumumab in the KMF database. Results: In this study, 196 patients were treated with daratumumab, lenalidomide, and dexamethasone (DLd) regimen and 128 patients were treated with daratumumab, bortezomib, and dexamethasone (DBd) regimen. The median age at treatment, number of prior treatment regimens and time-to-next-treatment (TTNT) were 68, 4 and 8.02 months, respectively. A multivariate analysis showed that the TTNT under the DLd regimen was longer with either higher monocyte counts (analysis 1), higher white blood cell (WBC) counts (analysis 2), lower β 2 microglobulin (B2MG < 5.5 mg/L) or fewer prior regimens (<4). No parameters were correlated with TTNT under the DBd regimen. Conclusion: We propose a simple scoring model to predict a durable effect of the DLd regimen by classifying patients into three categories based on either monocyte counts (0 points for ⩾200/μl; 1 point for <200/μl) or WBC counts (0 points for ⩾3500/μl; 1 point for <3500/μl) plus B2MG (0 points for <5.5 mg/L; 1 point for ⩾5.5 mg/L). Patients with a score of 0 showed significantly longer TTNT and significantly better survival compared to those with a score of 1 or 2 (both p < 0.001). To confirm this concept, our results will need to be validated in other cohorts.
In this study, we analyzed the complete mitochondrial genome of the invasive Asian hornet Vespa velutina from Japan. The mitochondrial genome of V. velutina was identified as a circular molecule of ...16,765 bp, similar to that in other hornet species. It was predicted to contain 13 protein-coding, 20 tRNA, and two rRNA genes, along with one A + T-rich control region. The initiation codons ATC was found in one, ATG in four, ATT in five, and ATA in three genes, while TAA was the termination codon in all these genes. The average AT content of 13 protein-coding was 82%.
J. Neurochem. (2012) 122, 883–890.
Amyloid β‐protein (Aβ) and α‐synuclein (αS) are the primary components of amyloid plaques and Lewy bodies (LBs), respectively. Previous in vitro and in vivo studies ...have suggested that interactions between Aβ and αS are involved in the pathogenesis of Alzheimer’s disease and LB diseases. However, the seeding effects of their aggregates on their aggregation pathways are not completely clear. To investigate the cross‐seeding effects of Aβ and αS, we examined how sonicated fibrils or cross‐linked oligomers of Aβ40, Aβ42, and αS affected their aggregation pathways using thioflavin T(S) assay and electron microscopy. Fibrils and oligomers of Aβ40, Aβ42, and αS acted as seeds, and affected the aggregation pathways within and among species. The seeding effects of αS fibrils were higher than those of Aβ40 and Aβ42 fibrils in the Aβ40 and Aβ42 aggregation pathways, respectively. We showed that Aβ and αS acted as seeds and affected each other’s aggregation pathways in vitro, which may contribute to our understanding of the molecular mechanisms of interactions between Alzheimer’s disease and LB diseases pathologies.
Previous studies have suggested that interactions between amyloid β‐protein (Aβ) and α‐synuclein (αS) are involved in the pathogenesis of Alzheimer’s disease (AD) and Lewy body diseases (LBD). Fibrils and oligomers of Aβ and αS acted as seeds, and affected the aggregations with each other. These may contribute to our understanding of the molecular mechanisms of interactions between AD and LBD.
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