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European Journal of Haematology

Alessio Mesini, Maura Faraci, Stefano Giardino, Erica Ricci, Sebastiano Barco, Giuliana Cangemi, Edoardo Lanino, Elio Castagnola
Invasive fungal diseases (IFD) are a major cause of morbidity and mortality in immunocompromised children, especially those undergoing allogeneic hematopoietic stem cell transplantation (HSCT) [1].Recently Vicenzi et al highlighted the role of posaconazole oral suspension in IFD prophylaxis and treatment in hemato/oncological children, reaching acceptable plasma levels in both groups [2].Although posaconazole is not approved in patients <18 years of age, it has been introduced in guidelines for IFD prophylaxis in children aged > 13 years [1]...
March 15, 2018: European Journal of Haematology
Giulia Ravasi, Sara Pelucchi, Gaia Buoli Comani, Federico Greni, Raffaella Mariani, Irene Pelloni, Silvia Bombelli, Roberto Perego, Donatella Barisani, Alberto Piperno
OBJECTIVE: During hypoxia hepcidin expression is inhibited to allow iron mobilization to sustain erythropoietic expansion. We analysed molecular mechanisms underlying hypoxia-induced hepcidin inhibition in an in vivo model of acute hypoxia. METHODS: Mice were kept under normal or hypoxic conditions for 6h and 15h and treated with α-PDGF-BB antibody or PDGF-BB receptor inhibitor. Blood, liver, spleen, and bone marrow were collected to extract RNA and protein or to quantify EPO and PDGF-BB...
March 15, 2018: European Journal of Haematology
Charikleia Kelaidi, Antonis Kattamis, Filia Apostolakou, Christos Poziopoulos, Christina Lazaropoulou, Polyxeni Delaporta, Ino Kanavaki, Ioannis Papassotiriou
BACKGROUND: Levels of the angiogenic cytokines Placental Growth Factor (PlGF) and soluble Fms-Like Tyrosine Kinase-1 (sFlt-1) and the angiogenic balance, expressed by sFlt-1/PlGF ratio, are perturbed in Sickle Cell Disease and iron overload, but they have not been evaluated in Non-Transfusion-Dependent Thalassemia (NTDT). PATIENTS AND METHODS: We measured levels of PlGF, sFlt-1 and vWF:antigen in patients with NTDT of beta thalassemia genotype, and correlated them with erythrocytic indices and markers of iron overload, inflammation and tissue hypoxia...
March 15, 2018: European Journal of Haematology
Chunyan Yue, Yingying Ding, Yang Gao, Li Li, Yan Pang, Zenghui Liu, Hang Zhang, Yang Xiao, Zujun Jiang, Haowen Xiao
OBJECTIVES: In patients with very severe aplastic anemia (VSAA), neutropenia is prolonged and persistent, resulting in refractory overwhelming infections. Hematopoiesis recovery is urgently needed. METHODS: Six patients with de novo VSAA lacking HLA-identical sibling donors and those who experienced refractory infections, underwent haploidentical related donor (HRD) hematopoietic stem cell transplantation (HSCT) as a first-line therapy. The conditioning regimen consisted of busulfan, cyclophosphamide and rabbit antithymocyte globulin...
March 12, 2018: European Journal of Haematology
Alexander Röth, Jaroslaw Maciejewski, Jun-Ichi Nishimura, Deepak Jain, Jeffrey I Weitz
OBJECTIVE: Paroxysmal nocturnal hemoglobinuria (PNH) is a severe, life-threatening disorder for which early diagnosis is essential. However, given the rarity of the disease and non-specificity of symptoms, correct diagnosis may be delayed or missed. While various hematologic guidelines note common signs and symptoms associated with PNH, international expert consensus based on real-world clinical experience and an actionable algorithm for non-specialists to facilitate screening and diagnosis are lacking...
March 12, 2018: European Journal of Haematology
Helena Claerhout, Sophie Van Aelst, Celine Melis, Thomas Tousseyn, Olivier Gheysens, Peter Vandenberghe, Daan Dierickx, Nancy Boeckx
OBJECTIVE: Diagnosing myeloid sarcoma remains challenging and we aimed to provide clinicopathological features to facilitate diagnosis. METHOD: Clinicopathological data from 41 patients with de novo and 31 with secondary myeloid sarcoma were reviewed. RESULTS: Most de novo cases presented with isolated myeloid sarcoma (n=19) or myeloid sarcoma with concurrent acute myeloid leukemia (n=15). Most secondary cases presented after acute myeloid leukemia (n=11), myeloproliferative neoplasm (n=9) or myelodysplastic syndrome (n=8)...
March 12, 2018: European Journal of Haematology
Krista Vaht, Magnus Göransson, Kristina Carlson, Cecilia Isaksson, Stig Lenhoff, Anna Sandstedt, Bertil Uggla, Jacek Winiarski, Per Ljungman, Mats Brune, Per-Ola Andersson
OBJECTIVES: Antithymocyte globulin (ATG)-based immunosuppression remains a cornerstone in aplastic anaemia (AA) treatment. However, most ATG studies are not population-based and knowledge about real-world results concerning response and outcome could offer important information for treating physicians. METHODS: We have recently performed a nationwide retrospective cohort study on all AA patients diagnosed in Sweden in 2000-2011 and now present treatment and outcome data on patients receiving first-line ATG...
March 12, 2018: European Journal of Haematology
Zachary Gross, Ashkon Rahbari, Eric Wirtschafter, Tanya M Spektor, Kyle A Udd, Sean Bujarski, Michael Ghermezi, Jason D Nosrati, Aleksandra Vidisheva, Benjamin Eades, Gary Cecchi, Tina Maluso, Regina Swift, James R Berenson
OBJECTIVE: To evaluate the efficacy and safety of elotuzumab and dexamethasone (Ed) for relapsed or refractory multiple myeloma (RRMM) patients. METHOD: This retrospective study evaluated the efficacy and safety of (Ed) treatment for 21 RRMM patients, 11 of whom were considered lenalidomide-refractory, and all of whom had progressed on at least 1 prior steroid-containing regimen. We also evaluated the efficacy of adding lenalidomide to a subset of patients following progression from Ed...
March 10, 2018: European Journal of Haematology
Laila Schneidewind, Thomas Neumann, Kai Alexander Probst, Christian Andreas Schmidt, William Krüger
OBJECTIVE: There is a substantial lack of data about men's health in adult allogeneic stem cell transplantation. METHODS: We conducted prospective unicentric non-interventional clinical study on men's health with a follow-up time of one year. RESULTS: Between 11/2013 and 12/2015, we were able to include 27 patients. AML was the most frequent underlying disease (25.9%), we mainly used intermediate intense conditioning protocols (77.8%). Erectile dysfunction, loss of libido and loss of efficiency were the most frequent symptoms of hypogonadism...
March 6, 2018: European Journal of Haematology
Tyler W Buckner, Michelle Witkop, Christine Guelcher, Robert Sidonio, Craig M Kessler, David B Clark, Wendy Owens, Neil Frick, Neeraj N Iyer, David L Cooper
INTRODUCTION: Health-related quality of life (HRQoL) is impaired in patients with hemophilia, however the impact in mild/moderate hemophilia B and affected women is not well characterized. OBJECTIVE: To evaluate factors that affect HRQoL in adults with hemophilia B and caregivers of affected children. METHODS: US adult patients and caregivers of affected children completed distinct ~1-hour online surveys including patient-reported outcome instruments...
March 5, 2018: European Journal of Haematology
Lei-Lei Zhou, Xiao-Yue Xu, Jie Ni, Xia Zhao, Jian-Wei Zhou, Ji-Feng Feng
OBJECTIVES: Due to the low incidence and the heterogeneity of subtypes, the biological process of T-cell lymphomas is largely unknown. Although many genes have been detected in T-cell lymphomas, the role of these genes in biological process of T-cell lymphomas was not further analyzed. METHODS: Two qualified datasets were downloaded from Gene Expression Omnibus database. The biological functions of differentially expressed genes were evaluated by gene ontology enrichment and KEGG pathway analysis...
March 5, 2018: European Journal of Haematology
Jackie Pratt, Philip Crispin
OBJECTIVES: To evaluate the dilute Russell viper venom time (DRVVT) for the detection of direct-acting oral anticoagulants (DOACs) and to investigate the effect of DOACS on coagulation assays. METHODS: Patients on DOACs and controls had plasma levels determined by an anti-Xa assay and dilute thrombin clotting time. Levels were correlated with the DRVVT as well as thrombin clotting time (TCT), prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen, protein C, protein S and antithrombin levels...
February 27, 2018: European Journal of Haematology
Bernardus G Goldman, Mark P Hehir, Sahr Yambasu, Edward M O'Donnell
Thrombocytopenia, defined as a platelet count less than 150,000 per microlitre, occurs in 7-12% of all pregnancies. Aside from anaemia, it is the most common haematological disorder in pregnancy. Despite its frequent occurrence, thrombocytopenia often leads to difficulties of diagnosis and management in pregnancy. Typically, a pregnant woman will have platelet counts of 150,000 to 450,000 per microlitre and platelet counts may be slightly lower than those of healthy, non-pregnant controls. Approximately 8% of pregnant women will develop mild thrombocytopenia (100,000-150,000 per microlitre) and while 65% of these women will have no underlying pathology, all pregnant women with platelet counts of less than 100,000 per microlitre should undergo further clinical and laboratory assessment...
February 21, 2018: European Journal of Haematology
Rasmus Tolstrup Larsen, Lars Hermann Tang, Nana Brochmann, Esben Meulengracht Flachs, Anne Illemann Christensen, Hans Carl Hasselbalch, Ann-Dorthe Zwisler
BACKGROUND: Patients with Philadelphia-chromosome negative Myeloproliferative Neoplasms (MPNs) report fatigue as the most common symptom and contributing significantly to reduction in their Quality of Life (QoL). Targeted non-pharmacological intervention to increase levels of physical activity is suggested as a fatigue-reducing and QoL-enhancing intervention in MPN patients. The interrelationship between physical activity, fatigue, and QoL have, to our knowledge, never been reported. METHODS: We analyzed data from 1,807 MPN patients...
February 21, 2018: European Journal of Haematology
Syed M Qadri, David A Donkor, Ishac Nazy, Donald R Branch, William P Sheffield
BACKGROUND: Surface desialylation is associated with erythrocyte aging and mediates phagocytic recognition and clearance of senescent erythrocytes. Neuraminidases, a family of glycohydrolytic enzymes, cleave the glycosidic linkages between sialic acid and mucopolysaccharides and have previously been implicated in erythrocyte dysfunction associated with sepsis. Erythrocytes in septic patients further display a phenotype of accelerated eryptosis characterized by membrane phospholipid scrambling resulting in phosphatidylserine (PS) externalization...
February 17, 2018: European Journal of Haematology
M Pick, V Vainstein, N Goldschmidt, D Lavie, D Libster, A Gural, S Grisariu, B Avni, D Ben Yehuda, M E Gatt
OBJECTIVE: Daratumumab is a promising new anti-myeloma agent. We report a single center "real world" series of multiple myeloma (MM) and amyloidosis (AL) patients treated with daratumumab. METHODS: Forty-one patients were included: 7 second line MM, 30 heavily pretreated (median number of therapies of 5) advanced MM, and 4 with AL. RESULTS: Second line patients and advanced AL showed high rate of durable overall responses. However, advanced MM patients had a dismal prognosis with an ORR of 36%, and a short median progression free and overall survival of 2...
February 17, 2018: European Journal of Haematology
Pavel Goriacko, Keith T Veltri
BACKGROUND: A complication of chronic liver disease (CLD) is the abnormality of coagulation. In clinical practice, this increased risk of bleeding has not been identified as a protective factor against stroke or systemic embolism associated with atrial fibrillation (AF). The objective of this study was to assess the safety of direct oral anticoagulant (DOAC) agents versus warfarin in CLD patients with AF. METHODS: This was a retrospective cohort study of patients with CLD and AF initiated on oral anticoagulants...
February 14, 2018: European Journal of Haematology
John Ashcroft, Ignacio Duran, Herbert Hoefeler, Vito Lorusso, Diana Lueftner, Marco Campioni, Michele Intorcia, Amit Bahl
OBJECTIVES: Patients with multiple myeloma (MM) often experience debilitating skeletal-related events (SREs: pathologic fracture, radiation to bone [RB], surgery to bone [SB] or spinal cord compression [SCC]). This is the first comprehensive, prospective, observational analysis of healthcare resource utilisation (HRU), independently attributed to SREs by investigators, in patients with MM. METHODS: Eligible patients had lytic bone lesions, life expectancy ≥6 months, Eastern Cooperative Oncology Group performance status ≤2, and ≥1 SRE in the 97 days before enrolment...
February 14, 2018: European Journal of Haematology
O W Bjerrum, J Samuelsson, W Ghanima, M Kauppila, C L Andersen
BACKGROUND: Patients with Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) have higher risks of developing thromboembolisms compared to the general population. International guidelines on the management of MPNs therefore include recommendations concerning thromboembolism prophylaxis. In clinical practice strict adherence to guidelines may be challenging and dependent on factors such as physician experience, outpatient clinic setting and access to therapy, however, no data exist on physician adherence or patient compliance to thromboembolism prophylaxis in MPNs...
February 10, 2018: European Journal of Haematology
Tadeusz Robak, Jerzy Blonski, Aleksander Bartłomiej Skotnicki, Magdalena Piotrowska, Tomasz Wróbel, Justyna Rybka, Janusz Kłoczko, Łukasz Bołkun, Bożena Katarzyna Budziszewska, Urszula Walczak, Anatoly Uss, Marta Fidecka, Piotr Smolewski
OBJECTIVES: PALG CLL4 is the first, randomized, phase IIIb study with cladribine, cyclophosphamide and rituximab (RCC) induction and subsequent maintenance with rituximab in previously untreated chronic lymphocytic leukemia (CLL) patients. METHODS: The induction treatment consisted of six RCC cycles regimen. Patients with complete response (CR) or partial response (PR) after an induction phase were randomized into a maintenance arm with rituximab or an observational arm...
February 10, 2018: European Journal of Haematology
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