High-dose therapy and autologous stem cell transplantation in patients with POEMS syndrome: a retrospective study of the Plasma Cell Disorder sub-committee of the Chronic Malignancy Working Party of the European Society for Blood & Marrow Transplantation

POEMS syndrome is a rare para-neoplastic syndrome secondary to a plasma cell dyscrasia. Effective treatment can control the diseaserelated symptom complex. We describe the clinical outcome of autologous stem cell transplantation for patients with POEMS syndrome, determining the impact of patient- and disease-specific factors on prognosis. One hundred and twenty-seven patients underwent an autologous stem cell transplantation at 1997-2010 with a median age of 50 years (range 26-69 years). Median time from diagnosis to autologous stem cell transplantation was 7.5 months with 32% of patients receiving an autologous stem cell transplantation more than 12 months from diagnosis. Engraftment was seen in 97% patients and engraftment syndrome was documented in 23% of autologous stem cell transplantation recipients. Hematologic response was characterized as complete response in 48.5%, partial response in 20.8%, less than partial response in 30.7%. With a median follow up of 48 months (95%CI: 38.3, 58.6), 90% of patients are alive and 16.5% of patients have progressed. The 1-year non-relapse mortality was 3.3%. The 3-year probabilities of progression-free survival and overall survival are 84% and 94%, respectively, with 5-year probabilities of progression-free survival and overall survival of 74% and 89%. In a cohort of graft recipients, detailed organ-specific symptom response demonstrated clear symptom benefit after autologous stem cell transplantation especially in relation to neurological symptom control. The data analysed in this study demonstrate the clinical utility of autologous stem cell transplantation for patients with POEMS syndrome

Allogeneic Stem Cell Transplantation for Myelodysplastic Syndrome Patients with a 5q Deletion

The deletion (5q) karyotype (del [5q]) in patients with myelodysplastic syndrome (MDS) is the most common karyotypic abnormality in de novo MDS. An increased number of blasts and additional karyotypic abnormalities (del [al]+) are associated with a poor outcome. We analyzed the outcome of allogeneic hematopoietic cell transplants (HCT) in patients suffering from MDS with only del (5q) or del (5q)+. A total of 162 patients, of median age 54 years (range, 9 to 73), having MDS and del (5q) abnormalities received HCT from identical siblings (n = 87) or unrelated donors (n = 75). The cumulative incidence of nonrelapse mortality and relapse incidence at 4 years was 29% (95% CI, 22 to 36) and 46% (95% CI, 38 to 54), whereas the estimated 4 year survival, relapse-free and overall, was 25% (95% CI, 18 to 33) and 30% (95% CI, 23 to 38), respectively. In a multivariate analysis patients with del (5q) and a blast excess displayed poorer survival (hazard ratio, 2.38; 95% CI, 1.44 to 3.93; PPeer reviewe

A Comparison of Blood Biochemical Parameters as a Tool to Measure Welfare in Free-ranging Red Deer (Cervus elaphus elaphus) after Chemical and Physical Restraint

Eighteen red deer were captured in box traps and then physically restrained in an immobilizing box; three red deer were captured twice, once by chemical restraint (anesthetized with a mixture of Xylazine, Tiletamine and Zolazepam) and once in the immobilizing box. It was found that microhaematocrit, total erythrocyte and leukocyte counts, as well as plasma cortisol, were higher after the physical restraint. Anserine and 3-methyl-L-histidine concentrations were higher after the physical restraint in animals undergoing both captures. Data supports previous studies indicating that the physical restraint after cap-ture is more stressful than the chemical one. Anserine and 3-methyl-L-histidine were higher after the physical restraint in red deer undergoing both methods, suggesting that measuring plasma aminoacyl–imidazole dipeptides and their components may be important in stressed animals prone to capture myopathy

When uncertainty generates more anxiety than severity: the prenatal experience with cystic adenomatoid malformation of the lung

Aim: To assess reasons for higher levels of anxiety generated by prenatal counselling of a condition with good outcome such as cystic adenomatoid malformation (CCAM) of the lung compared to a life-threatening malformation such as congenital diaphragmatic hernia (CDH). Materials and methods: The Spielberger State-Trait Anxiety Inventory (STAI-S) was used to measure anxiety in two groups of mothers carrying a fetus with the respective malformation. Results: Forty-four mothers completed the questionnaire (CCAM, n=21 and CDH, n=23). Before consultation, the mean STAI-S scores in the CCAM group (44.80±5.92) and in CDH group (44.05±4.96) were not significantly different but was significantly reduced in both groups after consultation (CCAM 44.80 vs. 41.60, P=0.014 and CDH 44.05 vs. 34.35, P=0.0001). The groups were not significantly different regarding gestational age at diagnosis. Conclusions: After initial prenatal counselling, uncertainty about prenatal outcome and lack of defined management plans in CCAM seems to be more important than higher mortality rate occurring in CDH.Peer Reviewe

Non-Destructive In Situ Investigation of the Study of a Medieval Copper Alloy Door in Canosa di Puglia (Southern Italy)

This paper reports the analyses carried out on the medieval copper alloy door (1111–1118 AD) of the mausoleum of Boemondo d’Altavilla in Canosa di Puglia (Southern Italy). The studied door is the smallest medieval bronze door extant in Italy and, unlike the other Byzantine doors, was most probably made in Canosa di Puglia and not in Constantinople. Analyses were performed to assess the chemical composition of the alloy patinas using a portable energy dispersive X-ray fluorescence (ED-XRF) instrument designed at the University of Salento. The experimental results suggested that the two door leaves have the same chemical composition, even if they appear different in both style and size. Furthermore, the alloy used for the door is different from the other previously-analyzed Byzantine bronze doors. The obtained results can be used in the future to compare the chemical composition of other Byzantine doors in order to better understand the manufacture of these precious artifacts

Non-Destructive In Situ Investigation of the Study of a Medieval Copper Alloy Door in Canosa di Puglia (Southern Italy)

This paper reports the analyses carried out on the medieval copper alloy door (1111–1118 AD) of the mausoleum of Boemondo d’Altavilla in Canosa di Puglia (Southern Italy). The studied door is the smallest medieval bronze door extant in Italy and, unlike the other Byzantine doors, was most probably made in Canosa di Puglia and not in Constantinople. Analyses were performed to assess the chemical composition of the alloy patinas using a portable energy dispersive X-ray fluorescence (ED-XRF) instrument designed at the University of Salento. The experimental results suggested that the two door leaves have the same chemical composition, even if they appear different in both style and size. Furthermore, the alloy used for the door is different from the other previously-analyzed Byzantine bronze doors. The obtained results can be used in the future to compare the chemical composition of other Byzantine doors in order to better understand the manufacture of these precious artifacts