Browsing by Subject "T-CELL LYMPHOMA"

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  • Palviainen, Mari; Laukkanen, Kirsi; Tavukcuoglu, Zeynep; Velagapudi, Vidya; Kärkkäinen, Olli; Hanhineva, Kati; Auriola, Seppo; Ranki, Annamari; Siljander, Pia (2020)
    Cancer alters cell metabolism. How these changes are manifested in the metabolite cargo of cancer-derived extracellular vesicles (EVs) remains poorly understood. To explore these changes, EVs from prostate, cutaneous T-cell lymphoma (CTCL), colon cancer cell lines, and control EVs from their noncancerous counterparts were isolated by differential ultracentrifugation and analyzed by nanoparticle tracking analysis (NTA), electron microscopy (EM), Western blotting, and liquid chromatography-mass spectrometry (LC-MS). Although minor differences between the cancerous and non-cancerous cell-derived EVs were observed by NTA and Western blotting, the largest differences were detected in their metabolite cargo. Compared to EVs from noncancerous cells, cancer EVs contained elevated levels of soluble metabolites, e.g., amino acids and B vitamins. Two metabolites, proline and succinate, were elevated in the EV samples of all three cancer types. In addition, folate and creatinine were elevated in the EVs from prostate and CTCL cancer cell lines. In conclusion, we present the first evidence in vitro that the altered metabolism of different cancer cells is reflected in common metabolite changes in their EVs. These results warrant further studies on the significance and usability of this metabolic fingerprint in cancer.
  • Molloy, K.; Jonak, C.; Woei-A-Jin, F. J. S. H.; Guenova, E.; Busschots, A. M.; Bervoets, A.; Hauben, E.; Knobler, R.; Porkert, S.; Fassnacht, C.; Cowan, R.; Papadavid, E.; Beylot-Barry, M.; Berti, E.; Violetti, S. Alberti; Estrach, T.; Matin, R.; Akilov, O.; Väkevä, L.; Prince, M.; Bates, A.; Bayne, M.; Wachsmuch, R.; Wehkamp, U.; Marschalko, M.; Servitje, O.; Turner, D.; Weatherhead, S.; Wobser, M.; Sanches, J. A.; McKay, P.; Klemke, D.; Peng, C.; Howles, A.; Yoo, J.; Evison, F.; Scarisbrick, J. (2020)
    Background Mycosis fungoides (MF) and Sezary Syndrome (SS) are the most common cutaneous T-cell lymphomas. MF/SS is accompanied by considerable morbidity from pain, itching and disfigurement. Aim To identify factors associated with poorer health-related quality of life (HRQoL) in patients newly diagnosed with MF/SS. Methods Patients enrolled into Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI; an international observational study in MF/SS) had their HRQoL assessed using the Skindex-29 questionnaire. Skindex-29 scores were analysed in relation to patient- and disease-specific characteristics. Results The study population consisted of 237 patients [60 center dot 3% male; median age 60 years, (interquartile range 49-70)], of whom 179 had early MF and 58 had advanced MF/SS. In univariate analysis, HRQoL, as measured by Skindex-29, was worse in women, SS, late-stage MF, those with elevated lactate dehydrogenase, alopecia, high modified Severity Weighted Assessment Tool and confluent erythema. Linear regression models only identified female gender (beta = 8 center dot 61; P = 0 center dot 003) and alopecia (beta = 9 center dot 71, P = 0 center dot 02) as independent predictors of worse global HRQoL. Item-level analysis showed that the severe impairment in symptoms [odds ratio (OR) 2 center dot 14, 95% confidence interval (CI) 1 center dot 19-3 center dot 89] and emotions (OR 1 center dot 88, 95% CI 1 center dot 09-3 center dot 27) subscale scores seen in women was caused by more burning/stinging, pruritus, irritation and greater feelings of depression, shame, embarrassment and annoyance with their diagnosis of MF/SS. Conclusions HRQoL is significantly more impaired in newly diagnosed women with MF/SS and in those with alopecia. As Skindex-29 does not include existential questions on cancer, which may cause additional worry and distress, a comprehensive validated cutaneous T-cell lymphoma-specific questionnaire is urgently needed to more accurately assess disease-specific HRQoL in these patients.
  • Trautinger, Franz; Eder, Johanna; Assaf, Chalid; Bagot, Martine; Cozzio, Antonio; Dummer, Reinhard; Gniadecki, Robert; Klemke, Claus-Detlev; Ortiz-Romero, Pablo L.; Papadavid, Evangelia; Pimpinelli, Nicola; Quaglino, Pietro; Ranki, Annamari; Scarisbrick, Julia; Stadler, Rudolf; Vakeva, Liisa; Vermeer, Maarten H.; Whittaker, Sean; Willemze, Rein; Knobler, Robert (2017)
    In order to provide a common standard for the treatment of mycosis fungoides (MF) and Sezary syndrome (SS), the European Organisation for Research and Treatment of Cancer-Cutaneous Lymphoma Task Force (EORTC-CLTF) published in 2006 its consensus recommendations for the stage-adapted selection of management options for these neoplasms. Since then, the understanding of the pathophysiology and epidemiology of MF/SS has advanced, the staging system has been revised, new outcome data have been published and novel treatment options have been introduced. The purpose of the present document is to update the original recommendations bearing in mind that there are still only a limited number of controlled studies to support treatment decisions for MF/SS and that often treatment is determined by institutional experience and availability. This consensus on treatment recommendations was established among the authors through a series of consecutive consultations in writing and a round of discussion. Recommended treatment options are presented according to disease stage, whenever possible categorised into first-and second-line options and supported with levels of evidence as devised by the Oxford Centre for Evidence-Based Medicine (OCEBM). Skin-directed therapies are still the most appropriate option for early-stage MF, and most patients can look forward to a normal life expectancy. For patients with advanced disease, prognosis is still grim, and only for a highly selected subset of patients, prolonged survival can be achieved with allogeneic stem cell transplantation (alloSCT). There is a high need for the development and investigation in controlled clinical trials of treatment options that are based on our increasing understanding of the molecular pathology of MF/SS. (C) 2017 The Authors. Published by Elsevier Ltd.
  • Boonk, Stephanie E.; Zoutman, Willem H.; Marie-Cardine, Anne; van der Fits, Leslie; Out-Luiting, Jacoba J.; Mitchell, Tracey J.; Tosi, Isabella; Morris, Stephen L.; Moriarty, Blaithin; Booken, Nina; Felcht, Moritz; Quaglino, Pietro; Ponti, Renata; Barberio, Emanuela; Ram-Wolff, Caroline; Jantti, Kirsi; Ranki, Annamari; Bernengo, Maria Grazia; Klemke, Claus-Detlev; Bensussan, Armand; Michel, Laurence; Whittaker, Sean; Bagot, Martine; Tensen, Cornelis P.; Willemze, Rein; Vermeer, Maarten H. (2016)
    Differentiation between Sezary syndrome and erythrodermic inflammatory dermatoses can be challenging, and a number of studies have attempted to identify characteristic immunophenotypic changes and molecular biomarkers in Sezary cells that could be useful as additional diagnostic criteria. In this European multicenter study, the sensitivity and specificity of these immunophenotypic and recently proposed but unconfirmed molecular biomarkers in Sezary syndrome were investigated. Peripheral blood CD4(+) T cells from 59 patients with Sezary syndrome and 19 patients with erythrodermic inflammatory dermatoses were analyzed for cell surface proteins by flow cytometry and for copy number alterations and differential gene expression using custom-made quantitative PCR plates. Experiments were performed in duplicate in two independent centers using standard operating procedures with almost identical results. Sezary cells showed MYC gain (40%) and MNT loss (66%); up-regulation of DNM3 (75%), TWIST1 (69%), EPHA4 (66%), and PLS3 (66%); and down-regulation of STAT4 (91%). Loss of CD26 (>= 80% CD4(+) T cells) and/or CD7 (>= 40% CD4(+) T cells) and combination of altered expression of STAT4, TWIST1, and DNM3 or PLS3 could distinguish, respectively, 83% and 98% of patients with Sezary syndrome from patients with erythrodermic inflammatory dermatoses with 100% specificity. These additional diagnostic panels will be useful adjuncts in the differential diagnosis of Sezary syndrome versus erythrodermic inflammatory dermatoses.
  • Knobler, R.; Berlin, G.; Calzavara-Pinton, P.; Greinix, H.; Jaksch, P.; Laroche, L.; Ludvigsson, J.; Quaglino, P.; Reinisch, W.; Scarisbrick, J.; Schwarz, T.; Wolf, P.; Arenberger, P.; Assaf, C.; Bagot, M.; Barr, M.; Bohbot, A.; Bruckner-Tuderman, L.; Dreno, B.; Enk, A.; French, L.; Gniadecki, R.; Gollnick, H.; Hertl, M.; Jantschitsch, C.; Jung, A.; Just, U.; Klemke, C. -D.; Lippert, U.; Luger, T.; Papadavid, E.; Pehamberger, H.; Ranki, A.; Stadler, R.; Sterry, W.; Wolf, I. H.; Worm, M.; Zic, J.; Zouboulis, C. C.; Hillen, U. (2014)
  • Boonk, Stephanie E.; Zoutman, Willem H.; Putter, Hein; Ram-Wolff, Caroline; Felcht, Moritz; Klemke, Claus-Detlev; Ranki, Annamari; Quaglino, Pietro; Whittaker, Sean; Bagot, Martine; Willemze, Rein; Vermeer, Maarten H. (2017)
  • Paediat IBD Porto Grp ESPGHAN (2018)
    Background: Risk benefit strategies in managing inflammatory bowel diseases (IBD) are dependent upon understanding the risks of uncontrolled inflammation vs those of treatments. Malignancy and mortality in IBD have been associated with disease-related inflammation and immune suppression, but data are limited due to their rare occurrence. Aim: To identify and describe the most common causes of mortality, types of cancer and previous or current therapy among children and young adults with paediatric-onset IBD. Methods: Information on paediatric-onset IBD patients diagnosed with malignancy or mortality was prospectively collected via a survey in 25 countries over a 42-month period. Patients were included if death or malignancy occurred after IBD diagnosis but before the age of 26years. Results: In total, 60 patients were identified including 43 malignancies and 26 fatal cases (9 due to cancer). Main causes of fatality were malignancies (n=9), IBD or IBD-therapy related nonmalignant causes (n=10; including 5 infections), and suicides (n=3). Three cases, all fatal, of hepatosplenic T-cell lymphoma were identified, all were biologic-naive but thiopurine-exposed. No other haematological malignancies were fatal. The 6 other fatal cancer cases included 3 colorectal adenocarcinomas and 3 cholangiocarcinomas (CCAs). Primary sclerosing cholangitis (PSC) was present in 5 (56%) fatal cancers (1 colorectal carcinoma, 3 CCAs and 1 hepatosplenic T-cell lymphoma). Conclusions: We report the largest number of paediatric-onset IBD patients with cancer and/or fatal outcomes to date. Malignancies followed by infections were the major causes of mortality. We identified PSC as a significant risk factor for cancer-associated mortality. Disease-related adenocarcinomas were a commoner cause of death than lymphomas.
  • Collin, P.; Vilppula, A.; Luostarinen, L.; Holmes, G. K. T.; Kaukinen, K. (2018)
    Background: The presenting symptoms of coeliac disease are often subtle and the diagnosis is frequently delayed or overlooked. Therefore, especially elderly patients may be denied the benefits conferred by gluten free diet which can be dramatically life-changing. Aim: To review the occurrence, clinical features, diagnosis and management in coeliac patients detected later in life. Methods: To review manuscripts concerned with coeliac disease in the elderly and to derive subgroups of elderly people from publications on the disorder. Results: Approximately a quarter of all diagnoses are now made at the age of 60 years or more and a fifth at 65 years or over. About 4% are diagnosed at 80 years or above. Around 60% remain undetected, since their symptoms are often subtle: tiredness, indigestion, reduced appetite. Good compliance with gluten free diet, resolution of symptoms and improvement in laboratory indices can be achieved in over 90% of patients. Conclusions: Coeliac disease not uncommonly presents for the first time in older patients and is an important diagnosis to make.
  • Salava, Alexander; Pereira, Pedro; Aho, Velma; Vakeva, Liisa; Paulin, Lars; Auvinen, Petri; Ranki, Annamari; Lauerma, Antti (2017)
    Staphylococcal enterotoxins have been shown to promote lymphoma-associated immune dysregulation. This study examined changes in the skin microbiome of parapsoriasis compared with intact skin. Swab microbiome specimens were taken of the parapsoriasis lesions of 13 patients. Control samples were taken from contralateral healthy sides of the body. Micro-biotas were characterized by sequencing the V1-V3 region of the 16S ribosomal RNA bacterial genes on the Illumina MiSeq platform. The most common genera in the microbiome data were Propionibacterium (27.13%), Corynebacterium (21.20%) and Staphylococcus (4.63%). Out of the Staphylococcus sequences, 39.6% represented S. epidermidis, with the rest including S. hominis, S. capitis and unidentified species. No significant differences were observed between the patients' parapsoriasis and contralateral healthy skin or between large-and small-plaque parapsoriasis. Notable interpersonal variation was demonstrated. These results suggest that parapsoriasis is not associated with significant alterations in the cutaneous bacterial microbiome.