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Background: Thromboses and phenotypic evolutions (leukemia, myelofibrosis) are the most frequent complications in polycythemia vera (PV) and essential thrombocythemia (ET). Aquagenic pruritus (AP) is not only PV symptom, but is also present in ET. The presence of pruritus in PV is associated with a lower risk of arterial thrombosis., Aims: To date, no equivalent study has been done to analyse the impact of AP for ET patients., Materials & Methods: We used the data from our cohort of patients with myeloproliferative neoplasms seen in our institution (OBENE database, NCT02897297). We collect information at diagnosis, presence or not of AP and all types of complications during their follow-up. To avoid masked PV, all JAK2 positive cases were tested isotopic red mass cell if appropriate., Results: Among 396 ET patients, presence of AP was found in 42 (10.6%). ET patients with AP were more proliferative, more symptomatic at diagnosis and more difficult to treat. Furthermore, they presented increased risk of thromboses (30.9 versus 17%, P = .03; OR = 2.2 [1.01;4.66]) and phenotypic evolutions (33.3 versus 13.3%, P = .0007; OR = 3.2 [1.44;6.77]), during follow-up., Discussion: Aquagenic pruritus is classically associated to PV. But we confirmed here that AP is also present in ET and characterizes patients with higher risk of morbidity (thrombotic events and phenotypic evolutions)., Conclusions: The systematic determination of the presence of AP in ET patients should permit us to better identify these high-risk patients for better management and follow-up., (© 2019 International Society on Thrombosis and Haemostasis.)

Background: The capillary zone electrophoresis method of albumin measurement is frequently used in monoclonal gammopathy patients but some studies suggest poor performances of the method in this population. The aim of this study was to analyse the impact of serum monoclonal immunoglobulins on human serum albumin determination by capillary zone electrophoresis method compared to other available methods., Method: We prospectively measured albumin in 100 freshly collected non-frozen serum samples in a monoclonal gammopathy patients population, by using four different methods: the capillary zone electrophoresis method, the bromocresol purple dye method, the nephelometric method and the turbidimetric method. Differences in albumin values between the different methods were analysed with respect to serum monoclonal immunoglobulin concentration. These differences were further investigated by measuring albumin levels in human serum samples spiked with exogenous monoclonal immunoglobulins., Results: Human serum albumin difference values between capillary zone electrophoresis compared to immunonephelometry method are significantly correlated with increasing monoclonal immunoglobulins concentrations: regression analyses revealed a correlation coefficient r 2  = 0.60 and a slope of 0.14 (0.12-0.17, 95% confidence interval). The capillary zone electrophoresis method overestimated serum albumin levels by up to 67% (12 g/L) when monoclonal immunoglobulin level was 63 g/L. The determination of albumin levels in human serum samples spiked with exogenous monoclonal immunoglobulins showed an overestimation of human serum albumin measurement by the capillary zone electrophoresis method proportional to the amount of monoclonal immunoglobulin added in the serum with a slope of 0.19 (0.18-0.20, 95% confidence interval)., Conclusion: Monoclonal immunoglobulins directly interfere with serum albumin measurement by the capillary zone electrophoresis method leading to a systematic overestimation of serum albumin concentrations proportional to the serum monoclonal immunoglobulin level., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: The capillary zone electrophoresis method of albumin measurement is frequently used for oncologic and haematologic patients but few data exist about the agreement between the albumin measurements performed by capillary zone electrophoresis and other methods. The aim of this study was to analyse the agreement between human serum albumin measurements by capillary zone electrophoresis and by the nephelometry, bromocresol purple and turbidimetry methods., Method: We prospectively measured 100 freshly collected non-frozen patient serum samples, by using four different methods: the capillary zone electrophoresis method performed with a CAPILLARYS 2 instrument, the bromocresol purple dye method performed with an Advia XPT analyser, the nephelometric method performed with a BN ProSpec analyser and the turbidimetric method with reagents from DiAgam and performed with the Advia XPT analyser., Results: A bias towards higher values in the lower range of albumin concentrations was observed with capillary zone electrophoresis compared to immunonephelometry: correlation coefficient r 2  = 0.925; slope of 0.86 (0.82-0.89, 95% confidence interval), which is significantly different from 1; and an intercept of 4.94 g/L (3.67-6.16, 95% confidence interval). Similar results were observed when comparing capillary zone electrophoresis to the bromocresol purple and immunoturbidimetry methods. The capillary electrophoresis method overestimated low albumin levels by up to 25% (5 g/L)., Conclusion: Compared to the nephelometry, turbidimetry and bromocresol purple methods, the capillary zone electrophoresis method tends to overestimate human serum albumin concentrations for levels below 30 g/L. This discrepancy could lead to an overestimation of the nutritional status, an inappropriate scoring of the disease and a delay in nutritional treatment., (Copyright © 2018 Elsevier B.V. All rights reserved.)

PAR2 activation in basal keratinocytes stimulates inflammation via the Ca 2+ -dependent production of mediators such as IL-1β, TNF-α, and TSLP. In this study, we investigated PAR2 calcium signaling and the consequent production of inflammatory mediators in differentiated human primary keratinocytes (DhPKs). Stimulation with the PAR2-activating peptide SLIGKV promoted Ca 2+ store depletion in both undifferentiated human primary keratinocytes and DhPKs. SLIGKV-evoked Ca 2+ store depletion did not trigger the store-operated Ca 2+ entry (i.e., SOCE) through ORAI1 in DhPKs compared with undifferentiated human primary keratinocytes. The inhibition of phospholipase C and the concomitant inhibition of TRPV1 and inositol triphosphate receptor in DhPKs abrogated the SLIGKV-evoked Ca 2+ store depletion; NF-κB activity; and the production of inflammatory mediators such as IL-1β, TNF-α, and TSLP. Taken together, these results indicate a key role for both InsP3R and TRPV1 in Ca 2+ internal stores in the PAR2-evoked Ca 2+ release and consequent skin inflammation in DhPKs. These findings may provide clues to understanding the pathological role of DhPKs in skin disorders in which PAR2 is known to be involved, such as atopic dermatitis, Netherton syndrome, and psoriasis., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

Unlabelled: The hook effect, which has long been detected and documented for immunoradiometric assays (IRMA) such as those measuring prolactin or thyroglobulin, occurs when the serum antigen level is extremely high, thus inducing a bias in the methodology of measurement., Results: We report the case of an 80-year-old man with confirmed medullary thyroid carcinoma (MTC). In the case reported here, the clinical status of the patient contrasts with his tumor antigen, serum calcitonin (CT), concentrations. The measured increased CT concentrations revealed the presence of a hook effect. This phenomenon occurs due to an excess of antigen during the one-step IRMA where the signal antibodies, bound to the non-captured antigens, are washed out during the measurement, inducing the loss of signal. Aiming to prevent the "hook effect", successive dilutions of the same sample of serum were done., Conclusions: Previous studies have shown when one-step IRMA reveals high concentrations of a tumor serum antigen (i.e. prolactin or thyroglobulin), a two-step IRMA or a systematic 1:10 dilution of the serum sample prevents the formation of the "hook effect". In our case report, the CT "hook effect" formation was prevented by performing serial dilutions of the serum sample., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background: Epidemiological studies have established that a low serum concentration of carotenoids was associated with risk of Age-Related Macular Degeneration (ARMD). The aim of this study was to determine carotenoid levels in serum and in different lipoprotein fractions in patients diagnosed for ARMD and in matched control group., Method: Thirty-four ARMD patients and 21 control subjects from Brest area (France) have been included to this study. Lipoproteins have been separated from serum by gradient density ultracentrifugation. We measured concentration of carotenoids and tocopherols in serum and in different lipoprotein fractions by HPLC., Results: No difference was observed between ARMD patients and control subjects in total serum carotenoids. Individual carotenoid levels showed that only lycopene was decreased significantly in serum, LDL and HDL fractions in patients (P<0.05). Concentrations in serum and lipoparticle fractions of lutein and zeaxanthin, the major pigments present in macula were not modified between both groups., Conclusions: Lycopene, as liposoluble antioxidant nutrient, is the only carotenoid altered in ARMD patients. It cannot be excluded that this effect is related to different dietary habits, but we hypothesise that lower lycopene status could result also from specific antioxidant protection of lutein and zeaxanthin by lycopene.