bluuebunny/crossref_metadata_2025_split huggingface dataset:
PB2262 HEALTHCARE RESOURCE UTILIZATION (HRU) ASSOCIATED WITH MINIMAL RESIDUAL DISEASE (MRD) STATUS IN ADULTS WITH B‐CELL PRECURSOR (BCP) ACUTE LYMPHOBLASTIC LEUKEMIA (ALL)
Authors: Pigneux A. , Montesinos P. , Cong Z. , Zhang X. , Pownell A. K. , Wieffer H. , McKendrick J. 
Published: June 2019

Background:The presence of MRD is the most important risk factor for relapse and mortality in patients with BCP ALL who achieve complete remission (CR). Given the poor outcomes associated with MRD‐positive (MRD+) ALL, achieving MRD‐negativity (MRD‐) is critical. However, the HRU associated with MRD status is unknown.Aims:To investigate the HRU associated with MRD status among adult patients with BCP ALL in first CR (CR1) in France, Germany, Italy, Spain and UK (EU5).Methods:A cross‐sectional survey of physicians who use MRD testing in adults with BCP ALL in the EU5 countries was used to estimate the HRU. Physicians must be board‐certified or specialized in hematology/hemato‐oncology, have ≥ 5 years’ experience with ALL, have seen ≥ 2 ALL patients in the last 12 months or ≥ 5 patients in the last 5 years, and have experience using MRD testing in clinical practice. Two to 4 physicians were recruited in each country. Each physician completed a web‐based questionnaire and was followed by a telephone interview. Descriptive analyses were conducted. Outpatient visits to healthcare professionals and hospital resource use for ALL patients in CR1 with or without MRD during an average period of 6 months (assuming no relapse or allogeneic hematopoietic stem‐cell transplant occurred) were reported for each country and across countries.Results:According to the survey, across the countries, patients with MRD+ ALL had an average of 9.2 (ranging 4∼12) visits to hematologist/oncologist, 1.4 (0∼2.5) visits to radiologist, 1.8 (0∼3) visits to other specialist physician and 2.6 (0∼4.5) visits to general physician during a 6‐months period, compared with 7.4 (1∼11), 1.1 (0∼2.3), 1.4 (0∼2.3) and 2.3 (0∼4.0) visits, respectively, in patients with MRD‐ ALL (Table 1). The average proportion of patients with at least one hospital admission in patients with or without MRD was 64.7% (ranging 40.0%∼83.3%) vs 39.3% (0∼83.3%). Of the patients with at least admission, patients with MRD+ ALL had an average of 3.2 (1.5∼4.3) admissions with a mean length of stay (LOS) of 13.2 (0.5∼19.8) days per admission, compared with 2.1 (0∼4.3) admissions and a mean LOS of 8.9 (0∼15.3) days per admission in patients with MRD‐ ALL (Table 2).Summary/Conclusion:From the physicians’ estimates of the HRU for patients with ALL in CR1 in the EU5 countries, presence of MRD+ is generally associated with higher use of outpatient visits to physicians across different specialties and with higher use of hospital resources (except Spain). The results should be interpreted with the limitation of small sample sizes in each country.image
PB2266 HOW TO IMPROVE HEMATOLOGIC REFERRAL? ANALYSIS AND RECOMMENDATIONS
Authors: Saraiva F. , Postolachi R. , Vicente M. I. , de Sousa A. Botelho 
Published: June 2019

Background:Incidental laboratorial abnormalities detected in asymptomatic persons are increasingly becoming a frequent motive for referral to a haematological consultation, overloading outpatient units that are run by small teams. These referrals, along with the subsequent acceptance by the triage manager and further haematological investigation by a haematologist, originate from the fear of missing incipient severe haemopathies.Aims:To evaluate the adequacy and the result of these referrals to ascertain the hypothesis that a large proportion of the cases have no indication for specialized haematological care.Methods:Retrospective analysis of patients referred to the Haematology consult of a central hospital over a 2‐year period (Jan/16‐Dec/17), excluding the ones who already had a known diagnosis. We evaluated the reason for referral, the final diagnosis and at a second stage we tried to quantify the referrals which could be managed in primary care, and their origin.Results:Out of 1162 evaluable patients (236 still in study), the majority (65%) came from Primary Health Care. The motive, in 85% of the cases, consisted in blood count abnormalities (59% cytopenias). In 13% of the cases, the initial abnormality no longer existed upon arrival. Finding an underlying pathology was possible in only 48% of the cases. From these, 14% could have been managed in Primary Health Care (e.g. iron deficiency; vitamin deficits) and 86% were haematologically relevant. As for the referrals which could have been avoided (no underlying haematological pathology or unconfirmed motive) the most frequent causes were cytopenias (anaemia 33%, leukopenia 15%, thrombocytopenia 12%) and they originated from Primary Health Care (72%).Summary/Conclusion:In this sample, we confirmed that a high proportion of referrals are manageable in Primary Health Care, particularly cytopenias, part of which are no longer present at the first haematological evaluation. The probability of a haematological investigation resulting in a clinically relevant diagnosis is very low, seemingly not justifying the consult time, the costs for the system or the personal costs for the patient. The elaboration of specifically designed recommendations for Primary Health Care and the implementation of interaction/discussion networks of pre‐triage cases are indispensable measures to optimize the haematological referral process.
PB2282 REASSESSING THE EFFICACY OF LOW DOSE RASBURICASE FOR MANAGEMENT OF TUMOR LYSIS SYNDROME IN HIGH RISK HEMATOLOGIC MALIGNANCIES
Authors: Heering G. , Loebstein R. , Rabinovitch H. Rotem , Avigdor A. , Merkel D. , Benjamini O. , Volchek Y. , Nagler A. , Canaani J. 
Published: June 2019

Background:Tumor lysis syndrome (TLS) is a potentially life‐threatening metabolic derangement seen in aggressive hematologic malignancies which is characterized by the massive release of toxic metabolic byproducts and consequent kidney damage. Whereas rasburicase is an established standard of care for management of TLS, the high cost of administering standard dosing of rasburicase has led to exploring lower doses of rasburicase.Aims:Assess our center's experience with lower dosing of rasburicase for management of TLS in aggressive hematologic malignancies.Methods:We studied all adult patients with hematologic malignancies who were treated with rasburicase in our institution between the years 2011‐2018. Charts were reviewed and demographic, clinical, and laboratory data were collected. Subjects were defined to be at high risk for TLS if they had any of the following features: acute leukemia, elevated uric acid levels at baseline (>7.5 mg/dL) or aggressive lymphoma.Results:Fifty‐five patients were evaluated. The median age of patients was 67 years (range 22‐92) consisting of 20 patients with acute leukemia, 21 with aggressive lymphoma, 11 with multiple myeloma, and 3 with other hematologic cancers. Nearly all analyzed patients (98%) were considered to be at high risk for TLS and 44 patients did meet criteria for clinical TLS. Twenty‐six patients (47%) received allopurinol prophylaxis. The median dose of rasburicase administered was 3 mg (range 1.5‐9) with 10 patients being given a second dose of rasburicase. The median baseline levels of uric acid and creatinine were 13.9 mg/dL (range 6.5‐42.8) and 2.18 mg/dL (range 0.63‐8.15), respectively. At 24 hours post administration of rasburicase the median uric acid levels were 7.9 mg/dL (range 0.8‐17.5) while at 48 hours they were 4.9 mg/dL (range 0.8‐13.5). Notably, 54 patients (98%) experienced a decrease in uric acid levels of at least 10% within the first 24 hours following rasburicase administration and 40% of patients had normal uric levels by that time. Within the initial 24 hours, creatinine levels were significantly improved in only a minority of patients with only 10 patients (18%) experiencing a decrease of more than 10% compared to baseline levels. Of note, 15 patients required dialysis during their admission and 26 patients died during the index hospitalization.Summary/Conclusion:The majority of subjects in our analysis of a high‐risk patient population responded favorably to low dose rasburicase in terms of rapid uric acid reduction although rates of dialysis and early mortality were substantial. Our data illustrate and confirm the feasibility of lower dosing of rasburicase to patients with aggressive hematologic malignancies.
PB2284 PSYCHOLOGICAL DISTRESS IN PATIENTS WITH HEMATOLOGICAL MALIGNANCIES: PREVALENCE AND ASSOCIATED FACTORS
Authors: Kacem K. , ghedira H. , sayadi M. , stambouli H. , eddhib J. , m'sadek F. , zriba S. 
Published: June 2019

Background:The diagnosis of hematological malignancy (HM) is associated with psychological suffering for patients who graft on their physical suffering. Psychological distress (PD) affects patient's quality of life and their ability to adhere to treatment.Aims:The exact prevalence of this event among patients with HM is not well specified. In this work, it is proposed to determine the prevalence of PD in patients with HM and the factors predicting its occurrence.Methods:We conducted a retrospective descriptive study that included 100 patients with HM followed in 2 Clinical Hematology departments in Tunis between 2010 and 2017. The DP was explored by the Distress Thermometer Scale (DTS) and by the validated Arabic version of the Hospital Anxiety and Depression Scale (HADS).Results:PD was detected in 40 patients byDTS and 43 patients by HADS.18 patients had an anxiety disorder alone, 5 patients had depressive disorder alone and 18 had concurrent anxiety and depression.DTS was significantly correlated with the global HADS, HADS‐depression and HADS‐Anxiety.In multivariate analysis, the associated comorbidities, the governorate of residence and the lack of complete response after treatment were predictive of PD occurrence.Summary/Conclusion:This study has shown a high prevalence of PD among patients with HM. Tunisian hematologists need to know how to detect these disorders as early intervention enhance their psychological wellbeing and improve their disease outcome.
PB2288 BENDAMUSTINE‐BORTEZOMIB‐DEXAMETHASONE (BVD) IN HEAVILY PRETREATED MULTIPLE MYELOMA: OLD/NEW ARM IN NOVEL AGENTS’ WORLD
Authors: Cerchione C. , Martinelli G. , Rocco S. , Palmieri S. , Nappi D. , Pareto A. E. , Pane F. , Catalano L. , Ferrara F. 
Published: June 2019

Background:Bendamustine is a bifunctional alkylating agent, with low toxicity, proved to be effective in relapsed, refractory and in new diagnosed Multiple Myeloma (MM).Aims:In this retrospective study, It has been evaluated efficacy and tolerance of Bendamustine, in combination with bortezomib‐dexamethasone (BVD) in patients with relapsed and refractory MM (rrMM), whose prognosis is particularly severe. A regional retrospective real‐life analysis of patients with rrMM who had been treated with BVD as salvage therapy has been performed.Methods:56 patients (31 M/25 F), with rrMM, median age at diagnosis 57.3 years (r. 36‐82), median age at start of treatment 61.8 years (r.37‐83) treated with several lines of treatments (median 6, r. 2‐11), every refractory to all the drugs previously received (also Bortezomib), received BVD (Bendamustine 90 mg/sqm days 1,2; Bortezomib 1.3 mg/sqm days 1,4,8,11, Dexamethasone 20 mg days 1,2,4,5,8,9,11,12, Pegfilgrastim day +4) every 28 days, until progression.ISS was equally distributed, and cytogenetic was evaluable in 12 patients, and in particular one del13q and one t (11;14). All the patients had previously been treated with schedule containing bortezomib and IMIDs, and 30% had also received radiotherapy. 67% of them had undergone at least to a single auSCT. All patients were relapsed and refractory to last therapies received before BVD.Results:Bendamustine was well tolerated, with grade 3 transfusion‐dependent anemia in 41% of patients, and 37% grade 3 neutropenia (no ospedalization was required, no septic shocks were observed). No severe extrahematologic toxicity was observed, only grade 1 gastrointestinal side effect (nausea), treated by common antiemetic drugs.According to IMWG, after a median follow‐up of 14 months (r.2‐36), ORR was 64% (36/56: 4 CR, 7 VGPR, 16 PR, 9 MR) with 8 PD and 12 patients in SD, which can be considered as an impressive result in this subset of rrMM patients. In particular, for 11 patients, BVD was, after having achieved at least a PR, a bridge to second auSCT, and for two patients a bridge to alloSCT. Three patients have shown a notable PR after failure of novel agents (i.e. Carfilzomib and Pomalidomide).Median time to response was 1.2 months (r.1‐3), median OS from diagnosis was 62.7 months (range 6‐151), median OS from start of Bendamustine was 9.8 months (range 2‐36).Summary/Conclusion:The triplet Bendamustine‐Bortezomib‐Dexamethasone has shown significant efficacy in a particularly severe setting of patients, relapsed and refractory to all available therapeutic resources, and, in particular cases, it could be considered as a bridge to a second autologous or allogenic SCT.
PB2289 HEALTH‐RELATED QUALITY OF LIFE AND OXYGEN TRANSPORT IN PATIENTS WITH NEPHROGENIC ANEMIA
Authors: Bondarchuk S. , Kovalev A. , Nikolay R. 
Published: June 2019

Background:End‐stage renal disease (EsRD) is often associated with an anemia. Most patients retrieve erythropoiesis stimulating agents, and so they presents with mild anemia. The tolerance of anemia of each patient is individual. Determination of venous blood gas (VBG) in conjunction with study of health‐related quality of life (HRQoL) can help in the individualization of treatment.Aims:To investigate the relationship between health‐related quality of life and venous blood gas in patients with nephrogenic anemia.Methods:30 patients with EsRD undergoing hemodialysis and receiving erythropoes stimulating therapy were involved in the study. 20 men, median age 52 years. Polycystic kidney disease was caused by the development of EsRD in 7 (23%) patients, diabetic nephropathy in 6 (20%) patients, glomerulonephritis also in 6 (20%) patients, nephrosclerosis in 5 (17%), and other causes in the remaining 6 patients (20%). HRQoL was studied using the FACT (Functional Assessment of Cancer Therapy Scale) questionnaire. Indicators of complete blood count, VBG were evaluated.Results:The hemoglobin median in the studied patients showed 105 g/l (81‐112 g/l), 26 patients experienced mild anemia (87%), 3 patients (10%) ‐ moderate anemia. in 1 patient there was no anemic syndrome. SvO2 median is 62.3%, while in only 18 (60%) patients the level of SvO2 is ≥60%. In general, the results of HRQoL analysis showed its deterioration in patients on all scales of the FACT questionnaire; the highest score was obtained in the scales “Physical well‐being” (Me 11 points) and “Anemia” (Me 30 points). When conducting a correlation analysis of hemoglobin and HRQoL, a statistically significant relationship was found on the scales “Physical well‐being” (r = ‐0.45; p < 0.05), “Emotional well‐being” (r = ‐0.39 p < 0, 05) and Anemia (r = ‐0.51; p < 0.05). A statistically significant correlation was obtained between the level of hemoglobin and the level of oxygen in venous blood. At the same time, no significant correlation was established between HRQoL and the saturation of venous blood (p > 0.2).Summary/Conclusion:According to the results of the HRQoL analysis, it was established that in patients with nephrogenic anemia, HRQoL decreases depending on the concentration of hemoglobin. The generally accepted target level of hemoglobin (100‐110 g/l) allows to avoid severe complications, but not in all cases it can adequately meet the need for O2 in patients. According to the data obtained, only 60% of patients achieved SvO2 ≥ 60%, which indicates the presence of tissue hypoxia.
PB2313 OCULAR MANIFESTATIONS OF SICKLE CELL DISEASE: A SINGLE CENTRE STUDY
Authors: Efobi C. , ejiofor O. , uzozie C. , onyiaorah A. 
Published: June 2019

Background:Sickle cell disease (SCD) is an inherited haemolytic disorder that affects virtually all body systems including the eye. Ocular complications of SCD could have dire consequences if not detected early.Aims:We aimed at determining the prevalence of ocular complications of SCD in our patients.Methods:This study was carried out on patients attending both the adult and Paediatric Sickle cell clinic at Chukwuemeka Odumegwu Ojukwu teaching Hospital, Akwa, Anambra State, Nigeria, over a 6 month period. A structured questionnaire was administered to consenting patients to determine their sociodemographic variables and other characteristics. Visual acuity was determined with multiple optotype Snellen's chart at 6 metres in natural daylight. Using pen‐torch, the face, external eye examination, anterior segment examination and pupillary reaction to light were assessed. Magnified anterior segment examination was performed with slit‐lamp biomicroscope. All subjects underwent dilated fundoscopy (after instillation of tropicamide 0.5% eye drop) using Welch Allyn indirect ophthalmoscope.Results were analysed using SPSS version 22.0 Inc Chicago Illinois and represented in tables. Statistically significant P value was set at < 0.05Results:A total of 28 patients with age range of 6‐42 years were seen. 17(60.7%) were males, while 11(39.3%) were females. 8(28.6%) had at least one ocular anomaly comprising of refractory errors 3(10.8%), sickle cell retinopathy 4(14.3%) and glaucoma 2(7.1%). Only 2(7.1%) routinely visit eye clinic. SCD retinopathy was found to increase with age (P value = 0.005)Summary/Conclusion:SCD retinopathy was found more in the older age group despite the small sample size at a prevalence rate of 14.3%. Early initiation of routine ocular examination for SCD patients will be beneficial to avert these complications. More efforts should be made at educating the patients. Larger studies will be required to determine the actual prevalence of ocular anomaly in SCD patients in Anambra State.
PB2315 ECHO HEART AND TISSUE DOPPLER ASSESSMENT AS EARLY PREDICTORS OF CARDIAC INJURIES IN SICKLE CELL PATIENTS
Authors: Sayed N. 
Published: June 2019

Background:Major cardiac abnormalities in patients with sickle cell disease are related to volume overload effects of chronic anemia and ferritin level. Patients with SCA have reduced LV global longitudinal strain. In the detection of subclinical LV dysfunction in patients presenting with sickle cell disease, 2D speckle tracking and tissue Doppler echocardiography appears to be useful to assess systolic dysfunction and diastolic dysfunction respectively.Aims:The aim of our study was to evaluate cardiac performance in adult Egyptian asymptomatic sickle cell patients.Methods:The study was conducted on 30 SCD patients who were recruited from the hematology clinic of Kasr El‐Ainy Hospital‐ Cairo University and 15 healthy control who were age and sex matched. Two‐dimensional speckle tracking echocardioghraphy (STE), M‐mode, Doppler and tissue Doppler echocardiography (TDE) were are used to assess systolic and diastolic dysfunction in both groups. We used parameters (GLS, AP3L, AP2L, AP4L, EF, LVIDS and LVIDd) for systolic dysfunction. And for diastolic dysfunction we used parameters (medial and lateral E/e’, E/A ratio, Dt and S velocity).Results:our results revealed significant increase in LVIDS and LVIDd dimensions in cases compared to control group (P < 0.001). None had an ejection fraction below 55%. Diastolic dysfunction was elicited in 8 patients (26%). There was a statistically significant correlation between the serum ferritin level and lateral E/E’ ratio. There was a significant reduction in GLS (p value.026) and also apical segments longitudinal strain (p value.002) compared to control. There was statistically significant negative correlation between number of sickle cell crises per year and GLS (p = 0.040). There was a statistical significant inverse correlation between Global LV systolic function and hemoglobin level (p = 0.04).Summary/Conclusion:Cardiac performance should include assessment of both systolic and diastolic parameters as there could be alteration in LV diastolic filling indices even in absence of systolic dysfunction. Hence these indices can be used as an early marker for cardiac affection.Speckle tracking echocardiography can recognize the cardiac involvement in really early stages and initiation of treatment and decreasing rate of painful crises to prevent irreversible myocardial dysfunction.
PB2316 EFFECT OF VITAMIN D SUPPLEMENTATION ON CLINICAL OUTCOMES OF SICKLE CELL DISEASE
Authors: Adly A. , Ebeid F. , Sallam S. , Gohar A. 
Published: June 2019

Background:Sickle cell disease (SCD) is one of the most common hereditary hemoglobinopathy. Vitamin D deficiency has emerged as a public health focus in recent years and it is one of the most common nutritional conditions among persons with SCD and there are characteristics specific to SCD that may contribute to this phenomenon including decreased appetite, inability to absorb nutrients due to damage to the intestinal mucosa, as well as an increased basal metabolic rate and higher nutritional demands to sustain normal physiologic functioning.Aims:Assess the level of vitamin D in pediatric SCD patients. Assess the effect of vitamin D supplementation on its clinical outcome including; frequency of vaso‐occlusive crisis and hospital admission and rate of infections.Methods:The current study is a follow‐up prospective study that included 50 children and adolescents with SCD recruited from the regular attendants Hematology/Oncology Clinic in Pediatric Hospital, Ain Shams University. Patients with SCD as confirmed by qualitative and quantitative analysis of hemoglobin using high performance liquid chromatography (HPLC) at their steady state were recruited. Patients with any inflammatory condition within one month prior to sample collection and those on vitamin D and/or calcium supplementation in the last 6 months were excluded. All patients subjected to clinical assessment and laboratory assessment including complete blood count, liver and kidney function tests and serum 25‐OH vitamin D level. Patients were divided into two groups according to their serum vitamin D level above and below 30 nmol and those with insufficient level were treated for three months and prospectively followed up for clinical outcome and had their vitamin D reassessedResults:There was a high prevalence of vitamin D deficiency (46%); 23 patients had vitamin D level less than 30nmol (Group 1) and 37 patients had vitamin D level more than 30 nmol (Group 2). Upon comparison between group 1 and group 2, there were no significant differences as regard clinic‐demographic data and treatment modalities. Patients with SCD and vitamin D deficiency had statistically significant higher history of fracture than those of with normal vitamin D level. There were statistically significant lower frequencies of joint and bone pain, sickle crisis, hospital admission and infections in patients with normal level of vitamin D. There was statistically significant improvement in vitamin D level after supplementation. There was statistically significant improvement as regard number of crisis after 3 months of vitamin D therapy (P = 0.043), with no statistically significant difference other studied parameters. There was statistically significant negative correlation between vitamin D level and number of crisis after 3 months of vitamin D therapySummary/Conclusion:The high prevalence of vitamin D deficiency in Egyptian SCD patients highlights the importance of early diagnosis and management on the clinical outcome of those patients