Amyloidosis associated with chronic lymphocytic leukemia.

Chronic lymphocytic leukemia (CLL), the most common form of leukemia in Western countries, rarely induces glomerular disease, but membranoproliferative glomerulonephritis or immunotactoid glomerulopathy has been reported. The proliferating cells in CLL are of mature B-cell origin and produce monoclonal immunoglobulin (Ig), thus leading to various kinds of autoimmune disorders or immunotactoid glomerulopathy. Although there have been a few reported cases of amyloidosis accompanying CLL, the type of amyloid fibrils has not been demonstrated nor described in detail, particularly regarding monoclonal Ig productivity. We report a rare case of amyloidosis associated with CLL, in which we detected ?-light chain type monoclonal Ig in the sera, urine, and on the surface membrane of lymphocytes, and discuss an association between monoclonal Ig-related disease and non-Hodgkin's lymphoma.     

Three-dimensional echocardiography could distinguish a ventricular septal defect adjacent to asymptomatic ruptured sinus of valsalva aneurysm

Sinus of Valsalva aneurysm (SVA) arises frequently in the right coronary sinus, and ventricular septal defect (VSD) is a prevalent coexistent cardiac abnormality. A 38-year-old asymptomatic male diagnosed with VSD on cardiac catheterization in his childhood, was referred to our hospital for the change in intensity of his cardiac murmur pointed out by his family physician. A grade V continuous murmur was auscultated with a thrill loudest at the forth left sternal border. Although, transthoracic and transesophageal echocardiography and cardiac catheterization have showed the ruptured right coronary sinus aneurysm, it was difficult to demonstrate coexistence of a doubly committed subarterial VSD. Three-dimensional echocardiography could provide clear images for diagnosis of the VSD closely adjacent to the ruptured SVA. The defect was confirmed at surgery.     

Intracontinental and intercontinental dissemination of Asian H5 highly pathogenic avian influenza virus (clade 2.3.4.4) in the winter of 2014–2015

Asian H5 highly pathogenic avian influenza viruses (HPAIVs) that possess the clade 2.3.4.4 HA gene have been identified in wild birds and poultry since late 2014 in both Europe and North America (N. America). Clade 2.3.4.4 H5 HPAIVs of the H5N8 subtype have been isolated in both regions, whereas reassortment viruses with NA N1 and N2 subtypes of the North American (N. American). avian lineage have only been identified in N. America. The HA genes of those isolates were closely related to genes of the HPAIVs that have caused massive outbreaks in poultry in Korea since January 2014. The outbreaks caused by those viruses and the genetic relatedness of their HA and NA genes are reviewed in this study. Although the illegal movement of poultry and poultry products cannot be ruled out as a cause of intercontinental and intracontinental dissemination of clade 2.3.4.4 H5 HPAIVs during the winter of 2014–2015, transmission of the viruses by infected migratory birds appears to be a more plausible mechanism for their dissemination. In particular, the involvement of migratory birds in HPAIV transmission between Asia and N. America is highly likely because of the reassortments between H5N8 HPAIV and the N. American lineage avian influenza viruses. Copyright © 2015 John Wiley & Sons, Ltd.     

Dominant formation of disulfidic linkages in the sulfur cross-linking reaction of isoprene rubber by using zinc stearate as an activator

A linear combination fitting in sulfur K-edge X-ray absorption near edge structure (S-XANES) measurements reveals each fraction of monosulfidic, disulfidic and polysulfidic linkages in solvent extracted sulfur cross-linked isoprene rubbers. The sulfidic linkage of a disulfidic type is found for the first time to be dominant when zinc stearate and N-(1,3-benzothiazol-2-ylsulfanyl)cyclohexanamine are used as the activator and accelerator, respectively, for the sulfur cross-linking reaction at 140 °C. The presence of the bridging bidentate zinc/stearate complex as an intermediate for the sulfur cross-linking reaction is supposed to induce the generation of abundant disulfidic linkages in the rubber networks. This unexpected observation is of use for the material design of high performance rubber products with anti-aging and thermal stabilities. S-XANES is a powerful tool that was used to reveal the characteristics of the sulfur cross-linking of rubber. These results will contribute to the development of rubber science and technology.

The bridging bidentate zinc/stearate complex, an intermediate for the sulfur cross-linking reaction, supposedly induces an abundance of disulfidic linkages in the rubber network.     

Whole-pelvic volumetric-modulated arc therapy for high-risk prostate cancer: treatment planning and acute toxicity

The objectives of this study were to evaluate dosimetric quality and acute toxicity of volumetric-modulated arc therapy (VMAT) and daily image guidance in high-risk prostate cancer patients. A total of 100 consecutive high-risk prostate cancer patients treated with definitive VMAT with prophylactic whole-pelvic radiotherapy (WPRT) were enrolled. All patients were treated with a double-arc VMAT plan delivering 52 Gy to the prostate planning target volume (PTV), while simultaneously delivering 46.8 Gy to the pelvic nodal PTV in 26 fractions, followed by a single-arc VMAT plan delivering 26 Gy to the prostate PTV in 13 fractions. Image-guided RT was performed with daily cone-beam computed tomography. Dose–volume parameters for the PTV and the organs at risk (OARs), total number of monitor units (MUs) and treatment time were evaluated. Acute toxicity was assessed using the Common Terminology Criteria for Adverse Events, version 4.0. All dosimetric parameters met the present plan acceptance criteria. Mean MU and treatment time were 471 and 146 s for double-arc VMAT, respectively, and were 520 and 76 s for single-arc VMAT, respectively. No Grade 3 or higher acute toxicity was reported. Acute Grade 2 proctitis, diarrhea, and genitourinary toxicity occurred in 12 patients (12%), 6 patients (6%) and 13 patients (13%), respectively. The present study demonstrated that VMAT for WPRT in prostate cancer results in favorable PTV coverage and OAR sparing with short treatment time and an acceptable rate of acute toxicity. These findings support the use of VMAT for delivering WPRT to high-risk prostate cancer patients.     

Synergistic effect of temperature and background counterions on ion-exchange equilibria

The effects of temperature and background counterions on ion-exchange selectivity for alkali metal ions and tetraalkylammonium ions on strongly acidic cation-exchange resins have been investigated using superheated water ion-exchange chromatography (SW-IEC). We have found out that alkali metal ions show reversal in the order of the distribution coefficient (K_D), from Li⁺ < Na⁺ < K⁺ < Rb⁺ in water at ordinary temperature to Rb⁺ < K⁺ < Na⁺ < Li⁺ in superheated water, when a relatively large cation such as cesium ion is used as the background counterion. The effect of counterion on the ion-exchange selectivity is enhanced with the ion-exchange resins of higher ion-exchange capacity and cross-linking degree. Tetraalkylammonium ions chosen as model ions for poorly hydrated ions also exhibit reversal in the order of K_D at around 430 K in superheated water. However, the effect of the nature of alkali metal counterions on the change in K_D values of tetraalkylammonium ions is rather small compared with the effect on the K_D of alkali metal ions. These results are attributed to the change in local hydration structures of the ions in the ion-exchange resin due to dehydration of alkali metal ions enhanced by interionic contacts of the analyte ion with the coexisting counterion and lower hydration energy of the ions at elevated temperatures. Although it has been considered that temperature is not effective at changing the ion-exchange separation selectivity, significant selectivity changes can be achieved by SW-IEC.

Metal ions in cation-exchange resins are dehydrated by interionic contacts with a co-existing counterion at elevated temperatures, which causes a drastic change in separation selectivity.     

The Effect of Intravenous Methylprednisolone on Recurrent Exacerbation in Hematologic Malignancy-associated Progressive Multifocal Leukoencephalopathy

We herein report a 65-year-old man with progressive multifocal leukoencephalopathy (PML) after 2-year remission from acute myeloid leukemia who developed recurrent episodes of left hemiparesis with gadolinium enhancement on magnetic resonance imaging. Steroid pulse therapy for each exacerbation induced clinical and radiological improvement, suggesting that exacerbations are an excessive immune response to the JC virus and distinct from immune reconstitution inflammatory syndrome (IRIS). Although glucocorticoids are recommended only for IRIS, steroid pulse therapy should be considered as a therapeutic option in cases of exacerbation of hematologic malignancy-associated PML. Importantly, neuroimaging is not sufficient to differentiate excessive inflammation from a controlled inflammatory response, for which steroids are not recommended.     

Diverse Pathological Findings of Interstitial Lung Disease in a Patient with Dyskeratosis Congenita

A 42-year-old man with a history of surgery for tongue cancer was referred to our hospital due to an abnormal chest shadow. High-resolution computed tomography showed lower lobe reticulation. A physical examination revealed nail dystrophy, oral leukoplakia, and reticulated hypopigmentation. Lung biopsy revealed subpleural and perilobular fibrosis, suggestive of usual interstitial pneumonia. However, multiple pathological findings, including homogenous fibrosis and cell infiltration in the centrilobular region, which were compatible with nonspecific interstitial pneumonia, and bronchiolitis were also seen. Genetic testing showed a hemizygous missense mutation in the DKC1 gene, and the patient was diagnosed with dyskeratosis congenita. Although anti-fibrotic therapy was initiated, the patient''s respiratory function has continued to decrease.