tbuckley/cabot-search huggingface dataset:
Early leukemic transformation of adult T-cell leukemia/lymphoma presenting as meningeal lymphoma
Authors: Barbara J. Bain
Journal: American Journal of Hematology (2016)

Adult T-cell leukemia/lymphoma (ATLL) presents as a leukemia in the majority of cases and as lymphoma in a minority. This patient was a 49-year-old AfroCaribbean man who had lived in the United Kingdom since the age of 17 years. He presented with multiple cranial nerve palsies and was found to have a minor degree of lymphadenopathy. Liver and spleen were not enlarged and there was no skin infiltration. His blood count and blood film were normal. Within three weeks his white cell count had risen to 12.1 × 109/l with a lymphocytosis. The hemoglobin concentration and platelet count remained normal. His blood film now showed pleomorphic lymphocytes; some were large with increased cytoplasmic basophilia while others had flower-shaped nuclei or bulbous nuclear lobulation (image). ATLL was suspected and this suspicion was increased by the presence of hypercalcemia (calcium 2.43 mmol/l). The provisional diagnosis was confirmed by demonstration of seropositivity for the human T-cell lymphotropic virus 1, by lymph node biopsy and by the immunophenotype. The lymphoma cells expressed CD2, CD5, CD16, CD25, and CD38; CD3 and CD7 were negative and CD4 and CD8 showed no clear-cut pattern of staining. Expression of CD25 is particularly characteristic of this diagnosis; CD38 is often also positive while CD7 is usually negative and CD3 may be downregulated (weak or absent membrane expression). As in a previously reported case 1, the nuclear shape was readily apparent in smear cells (lower right).
PTU-130 The Contribution of Gastroesophageal Reflux in Supragastric Belching
Authors: J Roscamp, J Patel, I Miah, T Wong, J Jafari
Journal: Gut (2016)

<h3>Introduction</h3> Supragastric belching (SGB) is characterised by rapid antegrade and retrograde flow of air in the oesophagus that does not reach the stomach.<sup>*</sup> Whilst proactive SGB is a behavioural phenomenon, reactive SGB occurs in response to a particular stimulus. Currently treatment for SGB is behavioural therapy however this may not be the most appropriate treatment for patients with reactive SGB. In this study we aimed to ascertain prevalence of proactive versus reactive SGB in response to a certain pathological stimulus i.e. GOR. <h3>Methods</h3> The database of the Oesophageal Physiology Laboratory at Guy’s Hospital London were retrospectively searched (November 2014 – present) for patients diagnosed with SGB (&gt;13 SGBs within 24 hrs). The 24 hour pH-impedance studies were analysed to differentiate proactive SGB from reactive (proactive SGB being an SGB without preceding reflux event (or preceding event lasting &lt;1 second), and reactive SGB being an SGB with preceding reflux event (&gt; 1 second)). A patient was then labelled as having predominantly reactive SGB (PR-SGB) if &gt;60% of their SGB was reactive and having predominantly proactive SGB (PP-SGB) if &gt;60% of their SGB was proactive. Reflux Diseases Questionnaire (RDQ) score was obtained for all patients. P value &lt;0.05 was considered significant. <h3>Results</h3> 28 patients identified for this study (14 males (M), 14 females (F), mean age 51 [27–75]). 82% of patients had PP-SGB events, 11% PR-SGB and 7% had the same number of proactive and reactive SGB. The most common symptoms in patients with PR-SGB were: heartburn 66.6%, belching 66.6% and throat burning sensation 66.6% and, in patients with PP- SGB: belching 68%, heartburn 39% and regurgitation 21%. In total, in all 28 patients, proactive SGBs accounted for 1124 and reactive 216 of SGB events (19.22% of all SGBs being reactive). The 3 patients with PR- SGB had an average RDQ of 3.53, whilst the average RDQ for the patients with PP- SGB was significantly lower 2.36 (p = 0.00004). There was 1 patient with 100% proactive SGB and no patient with purely reactive SGB. The median presentation of reactive SGB was 15.38%. <h3>Conclusion</h3> Treating GORD may resolve SGB in a small group of patients and ameliorate potential psychological stress caused by a behavioural therapy referral. Whether the remaining SGB events are purely behavioural or in reaction to other types of stimuli other than GOR requires further investigation. Having a high RDQ score may help in identifying patients with PR-SGB. <h3>Reference</h3> * Bredenoord AJ, <i>et al</i>. Aerophagia, gastric, and supragastric belching: a study using intraluminal electrical impedance monitoring. <i>Gut</i> 2004:<b>53</b>(11),1561–1565. <h3>Disclosure of Interest</h3> None Declared
Evidence of the Immune Relevance of <i>Prevotella copri</i>, a Gut Microbe, in Patients With Rheumatoid Arthritis
Authors: Annalisa Pianta, Sheila Arvikar, Klemen Strle, Elise E. Drouin, Qi Wang, Catherine E. Costello, Allen C. Steere
Journal: Arthritis & Rheumatology (2016)

Objective Prevotella copri , an intestinal microbe, may overexpand in stool samples from patients with new‐onset rheumatoid arthritis (RA), but it is not yet clear whether the organism has immune relevance in RA pathogenesis. Methods HLA–DR–presented peptides (T cell epitopes) from P copri were sought directly in the patients' synovial tissue or peripheral blood mononuclear cell (PBMC) samples using tandem mass spectrometry. The antigenicity of peptides or their source proteins was examined in samples from the RA patients or comparison groups. T cell reactivity was determined by enzyme‐linked immunospot assay; antibody responses were measured by enzyme‐linked immunosorbent assay, and cytokine/chemokine determinations were made by bead‐based assays. Serum and synovial fluid samples were examined for 16S ribosomal DNA for P copri using nested polymerase chain reaction analysis. Results In PBMCs, we identified an HLA–DR–presented peptide from a 27‐kd protein of P copri ( Pc ‐p27), which stimulated Th1 responses in 42% of patients with new‐onset RA. In both new‐onset RA patients and chronic RA patients, 1 subgroup had IgA antibody responses to either Pc ‐p27 or the whole organism, which correlated with Th17 cytokine responses and frequent anti–citrullinated protein antibodies (ACPAs). The other subgroup had IgG P copri antibodies, which were associated with Prevotella DNA in synovial fluid, P copri –specific Th1 responses, and less frequent ACPAs. In contrast, P copri antibody responses were rarely found in patients with other rheumatic diseases or in healthy controls. Conclusion Subgroups of RA patients have differential IgG or IgA immune reactivity with P copri , which appears to be specific for this disease. These observations provide evidence that P copri is immune‐relevant in RA pathogenesis.
Network visualization for outbreak response: Mapping the Ebola Virus Disease (EVD) chains of transmission in N'Zérékoré, Guinea
Authors: C. Valencia, H. Bah, B. Fatoumata, G. Rodier, B. Diallo, M. Koné, C. Giese, L. Conde, E. Malano, T. Mollet, J. Jansa, D. Coulombier, B. Sudre
Journal: Journal of Infection (2016)
Chromosome 17 Abnormalities Are Associated with Worse Overall and Relapse Free Survival in Patients with Acute Myelogenous Leukemia and Poor-Risk Cytogenetics.
Authors: Gautam Borthakur, Constantine Tam, Hagop Kantarjian, E. Lin, Jorge Cortes, Susan O’Brien, Charles Koller, Sherry Pierce, Michael Keating
Journal: Blood (2008)
Retention but Significant Reduction of BCR-ABL Transcript in Hematopoietic Stem Cells in Chronic Myelogenous Leukemia after Imatinib Therapy.
Authors: Akihiro Abe, Yosuke Minami, Kunio Kitamura, Fumihiko Hayakawa, Yuka Nomura, Makoto Murata, Akira Katsumi, Hitoshi Kiyoi, Catriona H.M. Jamieson, Jean Y.J. Wang, Tomoki Naoe
Journal: Blood (2008)
Targeting the KIT Activating Switch Control Pocket: A Novel Mechanism to Inhibit Mast Cell Activation and KIT D816V Neoplastic Mast Cell Proliferation
Authors: Geethani Bandara, Yun Bai, Eunice Ching Chan, Irina Maric, Olga Simakova, Scott C. Wise, Daniel Flynn, Dean D Metcalfe, Alasdair M Gilfillan, Todd M Wilson
Journal: Blood (2011)
Endothelial Progenitor Cells (EPC) with Colony Forming Capacity Are Derived from the Monocytic-Macrophage Lineage.
Authors: Natalia Lopez-Holgado, Mercedes Alberca Graduate, Eva M. Villaron, Julia Almeida, Alejandro Martin, Adriana Armellini Graduate, Fermin Sanchez-Guijo, Carmen Garcia, Belen Blanco, Ignacio Sanchez-Abarca, Jose A. Perez-Simon, Ramon Garcia-Sanz, Jesus F. San Miguel, Consuelo del Canizo
Journal: Blood (2005)
Double Cord Blood Units Allogeneic Transplantation for Hematological Malignancies: Study of the “3 Partners” Matching Variables on Transplant Outcomes
Authors: Mauricette Michallet, Mohamad Sobh, Stephane Morisset, Helene Labussiere, Marie Y. Detrait, Franck E. Nicolini, Sophie Ducastelle, Fiorenza Barraco, Xavier Thomas, Nathalie Tedone, Youcef Chelghoum, Jihane Fattoum, Isabelle Mollet, Valerie Dubois
Journal: Blood (2011)
Cardiac Iron Overload Assessed by T2* Magnetic Resonance (CMR) In Thalassemia Major Patients Treated with Different Iron Chelators
Authors: Elena Cassinerio, Alberto Roghi, Patrizia Pedrotti, Francesca Brevi, Laura Zanaboni, Giovanna Graziadei, Angela Milazzo, Maria Domenica Cappellini
Journal: Blood (2010)
THU0238 How To Diagnose and Prevent The Episodes of PFAPA Syndrome
Authors: M. Gunes, S. Cekic, S.S. Kilic
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most frequent repetitive fever syndrome in childhood. It is characterized with fever episodes lasting for about three to six days, once every three to eight weeks. <h3>Objectives</h3> In this study, we aim to elucidate the clinical clues that will facilitate the identification of PFAPA which is a clinical diagnosis and the efficacy and safety of colchicine prophlaxis in a large cohort. Additionally, we intend to investigate the relation between MEFV gene mutations and clinical findings of PFAPA. <h3>Methods</h3> Clinical and laboratory data of 400 consecutive patients with PFAPA syndrome followed up at the department of Pediatric Immunology and Rheumatology Uludag University Faculty of Medicine, were collected from the medical records retrospectively. <h3>Results</h3> Four-hundred children (256 male, 144 female), the mean age at diagnosis was 4.2 ± 2.2 years, were involved in our study. During the episodes, the mean number of WBC was high (12724,7/mm<sup>3</sup>) with predominantly neutrophils. The mean number of monocytes was found to be 1256.2/mm<sup>3</sup> and 90.2% of the patients had monocytosis. Serum amiloid A and C reactive protein levels were high in 84.6% and 77.8% of the patients, respectively. Procalcitonin was negative in 97.3% of the patients. MEFV gene heterozygous mutation was determined in 57 of the 231 patients (24.7%) tested. Colchicine prophylaxis was administered in 356 patients. The mean duration between episodes was prolonged from 18.8 to 49.5 days by prophylactic colchicine therapy. During the episodes, a single dose of 1 mg/kg methyl prednisolone was prescribed. Four patients underwent tonsillectomy. <h3>Conclusions</h3> Our study, include the largest number of patients with PFAPA syndrome in the literature. It is particularly important to assess and to demonstrate the high rate of response to colchicine prophylaxis in patients with PFAPA syndrome, especially those with MEFV variant. In blood screening neutrophilia associated with monocytosis and low procalcitonin level could contribute to diagnosis. <h3>References</h3> Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 1987; 110:43- 6. Frenkel J, Kuis W. Overt and occult rheumatic diseases: the child with chronic fever. Best Pract Res Clin Rheumatol 2002; 16:443- 69. John CC, Gilsdorf JR. Recurent fever syndrome in children. Ped Infect Dis J 2002; 21:1071 Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr 1999; 135:15- 21. Scholl PR. Periodic fever syndromes. Curr Opin Pediatr 2000; 563- 6. Padeh S, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome. J Pediatr 1999; 135:98- 101. <h3>Disclosure of Interest</h3> None declared
Low CLIP Expression On Leukemic Blasts From AML Patients Is Essential for the Generation of Effective CD4+ T Cell Responses.
Authors: Marvin M. van Luijn, Martine E.D. Chamuleau, James A. Thompson, Suzanne Ostrand-Rosenberg, Theresia M. Westers, Yuri Souwer, Gert J. Ossenkoppele, S. Marieke van Ham, Arjan A. van de Loosdrecht
Journal: Blood (2009)
Effects of Small-Molecule Inhibitors of TNFR2 Signaling Pathways On TNF-Mediated Expansion of CD4+FoxP3+ Regulatory T Cells
Authors: Xueqiang Wu, Yan Chen, Chunsheng Han, Yiwen Gong, Dingfang Bu, Yin Zhang, Junyan Xia, Qian Liu, Xue Chen, Yang Hu, Ping Zhu
Journal: Blood (2012)
SP0056 Beyond EMEUNET: Development of A European-Wide Network of Young Clinicians from Different Specialties Focusing on Inflammatory Diseases: Experiences from The Young Nephrologists' Platform
Authors: A.C. Ferreira
Journal: Annals of the Rheumatic Diseases (2016)

The Young Nephrologists9 Platform (YNP) was developed in 2012 and started its formal activity in 2013. It aims to involve all young professionals with interest in nephrology in all <i>European Renal Association - European Dialysis and Transplant Association</i> (ERA EDTA) activities. It is composed of 9 Board Members: 6 elected members from the ERA EDTA plus 3 ex-officio members, winners of the annual <i>Young Investigator</i> award. All young (&lt;40 years old) ERA EDTA members can apply to became Ordinary Members, and those are the ones who benefit from YNP activities. The communication among the members is performed by electronic means, as blast emails, surveys, and using the YNP blog. In these last three years, YNP developed two programs (free membership program and the advisory program), annually prepares the annual session at the European congress and annual CME Courses, and developed a new educational tool – the hot topics. The Free Membership Program offers 50 ERA EDTA memberships to YNP Ordinary Members, based on two types of accomplishments: the abstract-based type (30 free memberships to the 30 best abstracts submitted to annual ERA EDTA Congress) and the paper-based type (20 free memberships for manuscripts published on ERA EDTA journals by YNP members as 1st or last author). The Advisory Program is a 9 to 12 month length unique opportunity to exchange experiences and knowledge between two participants, the advisor (an experienced nephrologist, member of ERA EDTA) and the advisee (a young professional, member of YNP). The YNP sessions at the ERA EDTA annual congress are organized to provide an opportunity for young recognized researchers to show their experiments and results. Every year the Young Investigator Awardee is also invited to lecture during the YNP session. The YNP CME Courses are focusing different topics each year. The <i>Hot Topics</i> is seen as a new tool of study, and was developed to help young physicians to gather the latest information on certain topics, but also to interact with renowned experts. On each topic, YNP invites an expert to discuss a recent and relevant paper about the topic, and all YNP members can participate and communicate with the expert, via blog, adding questions or comments. Additionally, YNP members can test their knowledge with the YNP quizzes we provide on each topic. <h3>Disclosure of Interest</h3> A. C. Ferreira Grant/research support from: From the Portuguese Society of Nephrology.
Redox Associated Gene Expression Predicts For Responses To The Pro-Oxidant Molecule Imexon In Relapsed and Refractory B-Cell Non-Hodgkin Lymphoma: Results Of a Multi-Center Phase II Study
Authors: Paul M. Barr, Margaret M. Briehl, Steven H Bernstein, Jonathan W. Friedberg, Andrea Baran, Derick R. Peterson, Daniel Oscar Persky, Carla Casulo, Megan M. Herr, Jamie Littleton, Erin Cebula, Mark Schwartz, Soham D. Puvvada, Robert T Dorr, Richard I Fisher, Thomas P. Miller
Journal: Blood (2013)
FRI0420 Plasma Calprotectin in SPA-Patients, A Biomarker for Peripheral Arthritis:
Authors: I.M. Hansen, G. Bakland, Ø. Førre
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Spondyloarthritis (Spa) is a rheumatic disease with axial and peripheral inflammatory arthritis. The disease is associated with psoriasis, inflammatory bowel disease and uveitis. Inflammatory biomarkers as Sedimentation rate (SR) and CRP ar not as well correlated with disease activity in Spa as it is in Rheumatoid arthritis. Calprotectin, mainly secreted by neutrophilic granulocytes is elevated in sites of active inflammation Serum or plasma Calprotectin is elevated in rheumatoid arthritis, SLE and in IBD <h3>Objectives</h3> To investigate Plasma Calprotectin in Spa patients from Rana, Norway. Is Calprotectin elevated in Spa compared to healthy relatives, and relatives with symptoms of Spa? Is Calprotectin associated to age and gender? Is Calprotectin correlated to body mass index, other markers of inflammation, disease manifestations? <h3>Methods</h3> Patients with spondyloarthritis, were recruited from hospital registers, family doctors and by advertisement in local newspaper. Clinical data, Crp and HLAB27 were collected, x-ray and mri of SI-joint was performed if the patient had inflammatory backpain. If they fulfilled the ESSG-critera for Spondylarthritis they were included. The first degree relatives of the included patients were contacted and asked for symptoms of synovitis or inflammatory backpain by questionnaire. Symptomatic relatives were investigated, and included if they fulfilled the ESSG-critera. 387 spondylarthrits patients were included. 273 patients had mri of SI-joints235 patients, 51 symptomatic relatives and 74 healthy relatives were tested for Plasma Calprotectin, by Statistic testing with SPSS Chi square test or Students T-test <h3>Results</h3> Calprotectin levels are not significantly different in men and women, in Spa-patients, relatives with symptoms and healthy relatives of Spa patients. In Spa-patients there is no difference in Calprotectin-levels in inflammatory back-pain, radiological sacroiliitis, sacroiliitis on mri, psoriasis, inflammatory bowel disease, acute uveitis, reactive arthritis and HLA B27 positivity. Calprotectin levels are correlated to other inflammatory markers: SR, Crp, Hb and Trc, The best correlation is with Crp. Calprotectin is correlated to swollen joint count but not to BASDAI, BASFI or MHAQ <h3>Conclusions</h3> Se-Calprotectin could be a valuable biomarker I Spa, especially in peripheral Spa with polyarthritis <h3>References</h3> Hansson C, Eriksson C, Alenius G-M. S-Calprotectin (S100A8/S100A9): A Potential Marker of Inflammation in Patients with Psoriatic Arthritis. Journal of Immunology Research. 2014;2014:696415. doi:10.1155/2014/696415. <h3>Acknowledgement</h3> CALPRO AS, Arnstein Arnebergsvei 30 1366 Lysaker, Norway for analysing Plasma Calprotectin <h3>Disclosure of Interest</h3> None declared
Unrelated Cord Blood Transplantation (UCBT) in Adult and Pediatric Acute Lymphoblastic Leukemia: Impact of Minimal Residual Disease on Relapse and Survival,
Authors: Veronika Bachanova, Michael J Burke, Claudio Brunstein, Michael R Verneris, Qing Cao, John E. Wagner, Daniel J. Weisdorf
Journal: Blood (2011)
Immune (Idiopathic) Thrombocytopenic Purpura (ITP): Treatment Pattern and Splenectomy Rate from a Multinational Prospective Observational Study
Authors: Francesco Rodeghiero, Axel C. Matzdorff, Tiziano Barbui, Jean- Francois Viallard, Naveed M. Chowhan, Robert Robles, Boriana Kamenova, Ze Cong, Robert Deuson, John J. Isitt
Journal: Blood (2008)
Zika related microcephaly may appear after birth, study finds
Authors: Michael McCarthy
Journal: BMJ (2016)

Infants exposed to the Zika virus in utero may develop Zika associated microcephaly and other neurological complications after birth, researchers from Brazil and the United States warn. “The absence of microcephaly at birth does not exclude congenital Zika virus infection or the presence of Zika-related brain and other abnormalities,” they wrote.

Their report, which appeared in the US Centers for Disease Control and Prevention’s Morbidity and Mortality Weekly Report ,1 described the cases of 13 infants born in …
Minimal Residual Disease in High and Very High Risk ALL (HR/VHR-ALL) at the End of Induction (EOI) in FRALLE 2000 B and T Protocols.
Authors: Jean-Michel Cayuela, Kheira Beldjord, Nathalie Grardel, Hélène Cavé, Marie-Françoise Auclerc, Anne Auvrignon, Thierry Leblanc, Yves Perel, Françoise Mechinaud, Claudine Schmitt, Virginie Gandemer, Pascale Schneider, Claire Berger, Xavier Rialland, Catherine Paillard, Odile Lejars, Gerard Michel, Guy Leverger, Elizabeth A. Macintyre, Andre Baruchel
Journal: Blood (2007)
Identification of a Novel IGHCδ-BACH2 Fusion Transcript In B-Cell Lymphoma/Leukemia and the Expression Analysis of BACH2 Related with Other B-Cell Differentiation-Associated Genes In B-Cell Malignancies
Authors: Satoru Kobayashi, Tomohiko Taki, Yoshiaki Chinen, Yasuhiko Tsutsumi, Muneo Ohshiro, Tsutomu Kobayashi, Yosuke Matsumoto, Junya Kuroda, Shigeo Horiike, Kazuhiro Nishida, Masafumi Taniwaki
Journal: Blood (2010)
Adding KIT Inhibitor Dasatinib (DAS) to Chemotherapy Overcomes the Negative Impact of KIT Mutation/over-Expression in Core Binding Factor (CBF) Acute Myeloid Leukemia (AML): Results from CALGB 10801 (Alliance)
Authors: Guido Marcucci, Susan Geyer, Weiqiang Zhao, Andrew J Caroll, Donna Bucci, Geoffrey L. Uy, William Blum, Timothy Pardee, Meir Wetzler, Wendy Stock, Jonathan E. Kolitz, Ann-Kathrin Eisfeld, Clara D. Bloomfield, Richard M. Stone, Richard A. Larson
Journal: Blood (2014)
Mitochondrial Atpif1 Regulates Heme Synthesis in Developing Erythroblasts
Authors: Dhvanit I. Shah, Naoko Takahashi-Makise, Jeffrey D. Cooney, Liangtao Li, Iman Schultz, Eric L. Pierce, Anupama Narla, Shilpa Hattangadi, Nathanial Langer, Wen Chen, Tamara A. Dailey, Slater N. Hurst, Danilo Faccenda, Jessica Wiwczar, Terrence C. Law, Guillaume Vogin, Carlo Brugnara, Yi Zhou, Diane M. Ward, Benjamin L. Ebert, Michelangelo Campanella, Nika Danial, Harvey F. Lodish, Harry A. Dailey, Jerry Kaplan, Barry H. Paw
Journal: Blood (2011)