tbuckley/cabot-search huggingface dataset:
PB2231 THE MYELOPROLIFERATIVE NEOPLASMS UNCLASSIFIABLE. OUR EXPERIENCE
Authors: V. Accurso, M. Santoro, A. D. Contrino, M. Sardo, A. Perez, F. Di Piazza, A. M. Florena, A. Russo, S. Siragusa
Journal: HemaSphere (2019)

Background: According to the WHO classification, the designation of myeloproliferative neoplasms unclassifiable (MPN‐U) should be used for cases with clinical, morphologic, and molecular features of MPN failing to meet the diagnostic criteria of a specific entity. Reported incidence of MPN‐U varies significantly in different studies with a range up to &gt; 20%. However, most studies show an incidence of 10–15% or even less. When the 2016 WHO criteria have been applied, the incidence is reduced to &lt;5%. Aims: In this report we study the clinical and laboratory characteristics of 31 consecutive cases of unclassifiable myeloproliferative disease afferent to the center for myeloproliferative diseases of our Hematology Unit. Methods: From 2010 to 2018 our center has collected 508 cases of Philadelphia‐negative Myeloproliferative Neoplasms (MPNs). Data from these patients were collected in a specific database and analysed with simple statistic tools. Results: Among these 508 patients, 31 (6.2%) had a diagnosis of MPN‐U, 16 male and 14 female. Median age at diagnosis was 60.9 years. 22/31 (70.9%) were JAK2 V617F positive, 3/31 were CALR mutated (0.96%), 2/31 (0.64%) MPL mutated and 6/31 (19.3%) resulted as triple negative (TN) for driver mutations. Data from the other groups of MPNs (PV, ET, overtMF and preMF) were analised as well and compared to the ones from the MPN‐U group. Summary/Conclusion: Chronic myeloproliferative diseases unclassifiable (MPN‐U) represent the 6.2% of the cases relating to our center. No significant difference at diagnosis has been highlighted regarding sex, age and mutational status between MPN‐U and MPN classified according to WHO 2016. The exact incidence of MPN‐U is unknown. It is probable that frequency varies significantly according to the experience of the diagnostician and the specific classification system and criteria used. It is possible that careful use of the WHO 2016 classification may significantly reduce the frequency of MPN‐U.
PB2256 DISSOLVED PSEUDO‐THROMBOCYTOPENIA BY USING EVIDENCE‐BASED RESEARCH
Authors: S. H. Chen, T. suen
Journal: HemaSphere (2019)

Background: Platelet clumping is a common laboratory phenomenon that causes pseudo‐thrombocytopenia (PTC), spurious thrombocytopenia due to persistence of antibody or agglutinins. Microscopic examination of the peripheral blood smear is of key importance to confirm the diagnosis and to avoid any inappropriate or dangerous clinical and therapeutic decision. How to dissolve platelet clumping and make sure correct platelet count is an important procedure. Ethylenediaminetetraacetic acid (EDTA) is a well‐known anticoagulant for the hematological tests (CBC). EDTA‐dependent pseudo‐thrombocytopenia is the most common causes of platelet clumping in medical laboratory. Aims: To dissolved platelet clumping ex vivo and correct platelet count in medical laboratory avoid further inappropriate treatment and waste of medical resource. Methods: We drew blood of the patient in three tubes containing three different types of anticoagulants; EDTA, Sodium Citrate and Heparin. The three blood samples were run in fully automated Hematology analyzer (SYSMEX XN‐3000).Three tubes also process in water bath at 37 °C 10 minute and vortex until run automated Hematology analyzer again. Results: 54 years old female patient was referred to our lab for investigations as a case of implicated thrombocytopenia. The CBC revealed normal parameters apart from a low platelet count; 35 x10^9/L. A blood smear was ordered and it revealed server platelet clumping. Using different types anticoagulants, EDTA, Citrate, heparin result similar platelet count and still reveal platelet clumping on blood smear. We further pre‐treated warm water bath(37 °C) plus vortex before analysis. Platelet count was counted 138 x10^9/L by automatic hematologic analyzer in pre‐warm blood specimen. The smear also checked by microscopy without phenomenon of platelet clumping. Summary/Conclusion: Platelet cold agglutinin is confirmed by pre‐warm blood specimen which is a rare cause in pseudo‐thrombocytopenia. We used methods of evidence‐based medicine (EBM) to find out patient with cold agglutinins that affect platelet counting.
Percutaneous sensitization is limited by in situ inhibition of cutaneous dendritic cell migration through skin-resident regulatory T cells
Authors: Sho Hanakawa, Akihiko Kitoh, Rintaro Shibuya, Teruki Dainichi, Takashi Nomura, Tetsuya Honda, Gyohei Egawa, Atsushi Otsuka, Saeko Nakajima, Mitsugu Fujita, Kenji Kabashima
Journal: Journal of Allergy and Clinical Immunology (2019)
Revisions of Gastric Bypass—A Moral Obligation
Authors: Michel Gagner
Journal: JAMA Surgery (2019)

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AB0291C REMISSION IN RHEUMATOID ARTHRITIS: CONSIDERING NEW CUT-POINTS OF COMPOSITE DISEASE ACTIVITY INDICES ACCORDING TO THE ABSENCE OF SYNOVITIS BY ULTRASONOGRAPHY
Authors: Ana Bertoli, Marcelo Audisio, Mariana Benegas, Maximiliano Bravo, Erika Catay, Tomás Cazenave, Claudia Hartvig, Paula Kohan, Josefina Marin, Victoria Martire, David Navarta, Javier Rosa, Marcos Rosemffet, Santiago Ruta, Lida Santiago, Ethel Saturansky, Juan Soldano, Walter Spindler, Johana Zacariaz
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Achieving the state of remission in RA according to currently available criteria does not entirely prevent patient from radiological progression. On the contrary, the presence of subclinical synovitis by means of ultrasonography (US) correlates with radiological progression. <h3>Objectives</h3> To stablish cut-off values of composite disease activity indices (DAS28-CRP, DAS28-ESR, CDAI and SDAI) that adjust better to remission according to the absence of synovitis by US. <h3>Methods</h3> Observational, cross-sectional study that included 126 patients with diagnosis of RA who were in remission or low disease activity (DAS-ESR ≤3.2). For each patient we calculated the DAS28-ESR, DAS28-CRP, CDAI, SDAI. For US assessment we used the 12 joint simplified score. We considered the state of remission by US if synovial hypertrophy (SH) ≤1 and power Doppler (PD) signal= 0 in all joints. The cut-off values of the disease activity indices to define remission according the proposed US definition of remission were determined by ROC curves. <h3>Results</h3> Patients had a median (ICR) DAS28-ESR of 2.32 (0.60), DAS28-CRP= 1.89 (0.55), CDAI= 3.56 (3.0) and SDAI= 3.0 (4.7). The sensitivity, specificity, positive and negative predictive value of each of the selected cut-off points for the DAS28-CRP, DAS28-ESR, CDAI and SDAI for the definition of remission are shown in the Table. <h3>Conclusion</h3> In this study we describe new cut points of the different composite disease activity indices for the definition of remission. These cut points improve the accuracy of the definition of remission. However, they differ scarcely from the reference values making its impact uncertain in the long term management of patients with RA. Longitudinal studies are, therefore, warranted in order to elucidate the performance of these new cut points. <h3>Reference</h3> [1] Aletaha D, Smolen JS. Clin Exp Rheumatol2006; 24(6Suppl 43): S-45-51. <h3>Disclosure of Interests</h3> None declared
PB2296 SURVEILLANCE BREAST SCREENING PROGRAMS FOR PATIENTS WHO UNDERWENT CHEST RADIATION THERAPY (LYMPHOMA SURVIVORS)
Authors: M. Moschetta, M. Telegrafo, N. Troiano, T. Perrone, S. Bambace, M. Piombino, G. Specchia, F. Gaudio
Journal: HemaSphere (2019)

Background: Women who underwent chest radiation therapy (CRT) during pediatric/young‐adult age have an increased breast cancer (BC) risk. The cumulative BC incidence in women who underwent CRT is 13‐20%, higher than the incidence observed in the young female general population. BC is diagnosed on average about 15 years after CRT at about 40 years of age, compared with about 61 years in the non‐exposed female general population. Aims: From January 2018 to December 2018, a group of consecutive 46 patients (45 females, 1 male), aged between 23 and 62 (mean 44), who underwent CRT at least in the previous 8 years, underwent breast clinical, ultrasound (US) and mammographic examinations. Methods: Breast density at US was evaluated according to the BIRADS lexicon (homogeneous‐fat; homogeneous‐fibroglandular; heterogeneous) and compared with a control group of healthy patients (n = 46) with similar demographic features. Any statistical significant difference was searched by t‐student test for unpaired samples. The incidence of breast cancer in the examined series was assessed. Results: Among the examined series, 10/46 (22%) patients with homogeneous, 20/46 (43%) with fibroglandular and 16/46 (35%) with heterogeneous density were found. In the control group, 8/46 (17%) patients with homogeneous, 21/46 (46%) with fibroglandular and 17/46 (37%) with heterogeneous density were found. No significant difference occurred among the two groups of patients (p &gt; 0.05). 4/46 (3 females, 1 male) patients were affected by breast cancer (invasive ductal carcinoma, n = 3; ductal carcinoma in situ, n = 1) with a 9% incidence. Summary/Conclusion: A surveillance breast imaging protocol should be strongly recommended to patients with a previous CRT. Breast cancer incidence seems to be higher compared with general population. No significant effect on US breast density seems to exist in CRT patients. Future research should be directed at examining the underlying cancer biology and etiology of treatment induced cancers, as well as inherent and treatment‐induced genetic susceptibility of HL survivors.
PF651 POMALIDOMIDE, CYCLOPHOSPHAMIDE AND DEXAMETHASONE IN RELAPSED/REFRACTORY MULTIPLE MYELOMA PATIENTS IN A REAL LIFE SETTING: A SINGLE CENTER RETROSPECTIVE STUDY
Authors: S. Trudel, B. Tessoulin, M. Jullien, N. Blin, T. Gastinne, B. Mahé, V. Dubruille, A. Bonnet, A. Lok, P. Chevallier, P. Peterlin, A. Garnier, T. Guillaume, A. Le Bourgeois, S. Le Gouill, P. Moreau, C. Touzeau
Journal: HemaSphere (2019)

Background: Pomalidomide dexamethasone (Pd) is a standard of care for relapsed and refractory multiple myeloma (RRMM) patients who received at least two prior lines of therapy, including both lenalidomide and proteasome inhibitor (PI). In the phase 3 trial MM‐003, patients randomized in the Pd arm achieved an overall response rate (ORR) of 31% and a median progression‐free‐survival (PFS) of 4 months. Similar results were confirmed in the STRATUS trial (MM‐010). In order to improve these results, the triplet oral combination Pomalidomide Cyclophosphamide and Dexamethasone (PCD) has been investigated. To the best of our knowledge, we still need “real life” data evaluating the efficacy and safety of the triplet PCD combination in relapsed MM patients. Aims: To evaluate efficacy and safety of PCD in unselected RRMM patients outside clinical trials. Methods: All consecutive patients treated in our institution between January 2015 to July 2018 were included in this retrospective institutional review board approved analysis if they fulfilled the following criteria: (i) diagnosis of multiple myeloma according to International Myeloma Working Group (IMWG) consensus criteria (ii) relapsed and/or refractory to at least two priors therapies, including lenalidomide and PI and (iii) treated with pomalidomide, cyclophosphamide and dexamethasone. Response to treatment was determined according to IMWG criteria. Progression‐free‐survival (PFS) and OS were estimated using the Kaplan‐Meier method. Results: 49 consecutive patients were included. The median age was 66 years, including eight patients (16%) above 75 years. Nine (18%) patients had high‐risk cytogenetic (t(4;14) or 17p deletion). The median number of prior therapies was 3 (range, 2–8) and all patients were previously exposed to proteasome inhibitors and lenalidomide. Thirty‐nine (80%) patients were refractory to lenalidomide. Twenty‐eight (57%) patients were refractory to PI. A double refractory status (lenalidomide and PI) was identified in 21 (43%) patients. The ORR was 76%, including 27% very good partial response or better. With a median follow‐up of 16 months, the median PFS was 7.3 months and the median OS was not reached. Regarding safety, most frequent toxicity was hematologic, including 37% grade 3–4 cytopenias. Deep venous thrombosis were reported in 4% of patients. Nine patients (18%) discontinued therapy due to adverse event. Summary/Conclusion: This real‐life study demonstrated that PCD is therefore a manageable, cost‐effective and oral triplet combination for unselected RRMM patients eligible to Pd.
Abstract 13614: Prognostic Value of Cardiorespiratory Fitness in Patients With Chronic Renal Dysfunction: The FIT (Henry Ford Exercise Testing) Project
Authors: Amjad Ahmed, Waqas T Qureshi, Clinton A Brawner, Steven J Keteyian, Jonathan K Ehrman, Roger S Blumenthal, Khurram Nasir, Michael J Blaha, Mouaz H Al-Mallah
Journal: Circulation (2015)

Background: Chronic kidney disease (CKD) is associated with high morbidity and mortality. We tested the hypothesis that cardiorespiratory fitness (CRF) retains incremental prognostic value over renal function, as measured by estimated glomerular filtration rate (eGFR). Methods: Our analysis included 50,121 patients (mean age 55 ± 12.6 years and 47.5% females) from The Henry Ford Exercise Testing Project (FIT project), who underwent exercise stress testing between 1991 and 2009. Patients with heart failure or missing relevant covariate data were excluded. The mean duration between stress testing and creatinine measurement was 30 days and eGFR was estimated using the Modification of Diet in Renal Disease equation. Treadmill stress testing used the standard Bruce protocol and CRF was estimated using metabolic equivalent of task units (MET). Patients were followed-up for all-cause mortality. Multiple nested Cox models were used to determine the incremental prognostic value of CRF over eGFR. Results: The magnitude of renal dysfunction was inversely associated with (level of CRF (mean METs achieved 9.1, 9.0, 7.5, 5.5 METS for Stage I, II, III and more than III CKD respectively, p&lt;0.0001). Over a median follow-up duration of 6.7 yr, (IQR 3.9 to 10 yr), 6,308 (12.6%) patients died. CRF was a strong, independent predictor of mortality (p &lt;0.001) and at every stage of renal function, improved CRF was associated with improved survival. (figure). Using Cox analysis, CRF added improved discriminatory ability beyond traditional risk factors and renal function: Area under the curve (AUC) 0.837 (95% CI 0.832 - 0.842) vs. 0.814 (95% CI 0.809 - 0.8198), respectively, p-value &lt;0.0001. Renal function did not add incremental prognostic value once CRF was accounted for. Conclusion: Regardless of renal function, CRF is an independent predictor of mortality. Fitness adds incremental prognostic value over renal function in this large diverse clinical cohort.
PS1258 CLONAL B‐CELL LYMPHOCYTOSIS OF MARGINAL ZONE ORIGIN (CBL‐MZ): CLINICAL SIGNIFICANCE OF ABSOLUTE CLONAL B CELL COUNTS
Authors: C. Kalpadakis, G. Pangalis, I. Konstantinou, S. Sachanas, P. Tsirkinidis, M. Moschogiannis, X. Yiakoumis, M. Siakantaris, P. Tsaftaridis, T. Iliakis, M. Befani, M. Dimopoulou, I. Asimakopoulos, A. Dimitrakopoulou, G. Butsikas, P. Korkolopoulou, F. Kontopidou, E. Koulieris, C. Pontikoglou, M. Ximeri, M. Psylaki, M. Roumelioti, D. Rontogiannis, H. Papadaki, P. Panagiotidis, T. Vassilakopoulos, M. Angelopoulou
Journal: HemaSphere (2019)

Background: CLL‐like MBL is divided into low and high count, with significant clinical and biological differences. No studies have been conducted so far in CBL‐MZ. Aims: In accordance to the proposed grouping of CLL‐like MBL, we aimed to investigate the clinical significance of such distinction in MZL‐like MBL. Methods: 95 consecutive CBL‐MZ cases were analyzed, with any number of circulating CD5‐ CBL, no B‐symptoms, organomegaly, lymphadenopathy, cytopenias or any other features indicating a known lymphoproliferative disorder. Patients were grouped in 3 categories: A. low count (CBL≤500/μL), B. high count (CBL &gt; 500/μL and ≤5000/μL) and C. frank lymphocytosis (CBL&gt;5000/μL). Using this subdivision a comparison of several laboratory and disease evolution parameters was performed. Results: 3/95 patients belonged to group A, 59 to group B and 33 to group C. The characteristics and outcome of the three groups are shown in table. Apart from WBC, ALC and CBL that differed significantly by definition, the only parameters that were statistically different between the three groups, were absolute neutrophil counts, CD11c expression, BM infiltration, and the frequency of evolution to SMZL. More specifically, the frequency of CD11c expression was highest in group C (68%) compared to group B (33%) and A (0%). Surprisingly, ANC were significantly higher with rising ALC. The percentage of BM infiltration differed significantly between the three groups and was highest in the lymphocytosis group (C); however, this difference was mainly due to the difference between group B and C. Notably, the three patients with low‐count MBL had BM infiltration between 30–40%, which was similar to the group C. There was a trend for a higher frequency for SMZL evolution in group C. Four out of 5 patients who evolved to SMZL belonged to the group C, whereas the remaining one patient had a low‐count MBL. At a median follow‐up time of 42 months, need of therapy, time to progression (TTP), time to treatment (TTT) and overall survival (OS) did not differ significantly between the three groups. Not CBL counts, but elevated LDH and BM infiltration≥50% were the only significant factors for TTT by multivariate analysis. Treatment due to cytopenias was more frequent in group B. Summary/Conclusion: CBL‐MZ patients have an excellent prognosis. The cut‐off of 5x10 9/ /L CBL does not seem to be the optimal threshold to distinguish prognostically different subgroups, with the exception of SMZL development. BM infiltration≥50% and elevated LDH are associated with shorter TTT. image
PB2085 ENVIRONMENTAL AND OCCUPATIONAL DETERMINANTS OF MYELODYSPLASTIC SYNDROMES: EXPOSURE, ASSOCIATION AND RISK: A CASE CONTROL STUDY FROM PAKISTAN
Authors: N. Anwar, A. Arshad, N. Fatima, S. Sharif, M. Zaidi, T. Shamsi
Journal: HemaSphere (2019)

Background: Myelodysplastic syndromes are heterogeneous group of haematopoietic stem cell disorders and have a variable reduction in the production of red cells, platelets, and mature granulocytes. Previous studies have emphasised the possible association of disease with environmental and occupational factors. Aims: In Pakistan and the surrounding regions, patients do not have access to prompt diagnostic and treatment facilities. Moreover, the characteristics of many patients, such as a low literacy rate, exposure to hazardous chemicals, inadequate access to milk and water, and habitual narcotics use, make them more vulnerable to diseases like MDS at an early age compared with patients in the western world. In this regard, we hypothesised to sketch a roadmap for our patients to identify risk factors in their daily routines and make them aware of related causal factors that ultimately could lead to life threatening disorders like MDS and thus minimise their disease burden. With this background and rationale, we conducted a case control study that evaluated environmental and occupational determinants as risk factors for MDS patients. To the best of our knowledge, this is the first study conducted in Pakistan in this context. Methods This was a case control study carried out at the National Institute of Blood Diseases and Bone Marrow Transplantation. The participants included 153 de novo MDS cases and 447 age and sex matched controls. Disease characteristics, sociodemographics and exposure to chemicals were collected through a questionnaire. The chi squared test was applied for finding associations between categorical variables, and binary logistic regression was applied to find odds ratios for significant variables. Results: Among the study participants ( n = 600), there was a significant association between cases and controls regarding marital status, type of job, educational status and arsenic (p &lt; 0.0001). There was also significant difference found in study group with language (p = 0.037) and benzene (p = 0.021). The association with residency in rural and urban areas, and milk and water source was not found significant. Summary/Conclusion: We concluded that people in developing countries such as Pakistan could be at higher risk than those in industrialized countries of getting diseases like MDS owing to the unrecognised exposure to perilous environmental and occupational factors. This study will be helpful to understand the aetiology of MDS in Pakistani population.