journal

British Journal of Haematology

journal
https://read.qxmd.com/read/31945802/mutation-enhanced-international-prognostic-systems-for-essential-thrombocythaemia-and-polycythaemia-vera
#1
Ayalew Tefferi, Paola Guglielmelli, Terra L Lasho, Giacomo Coltro, Christy M Finke, Giuseppe G Loscocco, Benedetta Sordi, Natasha Szuber, Giada Rotunno, Annalisa Pacilli, Curtis A Hanson, Rhett P Ketterling, Animesh Pardanani, Naseema Gangat, Alessandro M Vannucchi
Survival prediction in essential thrombocythaemia (ET) and polycythaemia vera (PV) is currently based on clinically-derived variables; we examined the possibility of integrating genetic information for predicting survival. To this end, 906 molecularly-annotated patients (416 Mayo Clinic; 490 University of Florence, Italy), including 502 ET and 404 PV, were recruited. Multivariable analysis identified spliceosome mutations to adversely affect overall (SF3B1, SRSF2 in ET and SRSF2 in PV) and myelofibrosis-free (U2AF1, SF3B1 in ET) survival; TP53 mutations predicted leukaemic transformation in ET; "adverse" mutations occurred in 51 (10%) ET and 8 (2%) PV patients...
January 16, 2020: British Journal of Haematology
https://read.qxmd.com/read/31945798/platelet-and-immune-characteristics-of-immune-thrombocytopaenia-patients-non-responsive-to-therapy-reveal-severe-immune-dysregulation
#2
Elena Monzón Manzano, María Teresa Álvarez Román, Raúl Justo Sanz, Ihosvany Fernández Bello, Diana Hernández, Mónica Martín Salces, Larissa Valor, Isabel Rivas Pollmar, Nora V Butta, Víctor Jiménez Yuste
Multifactorial mechanisms leading to diminished platelet counts in immune thrombocytopaenia (ITP) might condition the ability of patients with ITP to respond to treatments. Examining their platelet and immune features, we aimed to detect singular characteristics of patients with ITP who do not respond to any treatment. We studied patients with chronic primary ITP who had been without treatment, or untreated (UT-ITP), for at least six months; included were responders to agonists of thrombopoietin receptors (TPO-RA), patients who showed no response to first- and second-line treatments (NR-ITP), and healthy controls...
January 16, 2020: British Journal of Haematology
https://read.qxmd.com/read/31943144/droplet-digital-pcr-is-a-sensitive-tool-for-the-detection-of-tp53-deletions-and-point-mutations-in-chronic-lymphocytic-leukaemia
#3
LETTER
Raffaele Frazzi, Veronica Bizzarri, Laura Albertazzi, Vincenza Ylenia Cusenza, Lia Coppolecchia, Stefano Luminari, Fiorella Ilariucci
No abstract text is available yet for this article.
January 14, 2020: British Journal of Haematology
https://read.qxmd.com/read/31943141/bcr-abl1-positive-acute-myeloid-leukaemia-relapsing-as-acute-lymphoblastic-leukaemia
#4
Xueyan Chen, Hongwei Chen
No abstract text is available yet for this article.
January 14, 2020: British Journal of Haematology
https://read.qxmd.com/read/31943135/upregulation-of-akr1c1-in-mesenchymal-stromal-cells-promotes-the-survival-of-acute-myeloid-leukaemia-cells
#5
Yajing Jiang, Ying Li, Jingying Cheng, Jiao Ma, Qinghua Li, Tianxiang Pang
The leukaemic bone marrow microenvironment, comprising abnormal mesenchymal stromal cells (MSCs), is responsible for the poor prognosis of acute myeloid leukaemia (AML). Therefore, it is essential to determine the mechanisms underlying the supportive role of MSCs in the survival of leukaemia cells. Through in silico analyses, we identified a total of 271 aberrantly expressed genes in the MSCs derived from acute myeloid leukemia (AML) patients that were associated with adipogenic differentiation, of which aldo-keto reductase 1C1 (AKR1C1) was significantly upregulated in the AML-MSCs...
January 14, 2020: British Journal of Haematology
https://read.qxmd.com/read/31944265/-90-y-ibritumomab-tiuxetan-in-patients-with-extra-nodal-marginal-zone-b-cell-lymphoma-of-mucosa-associated-lymphoid-tissue-malt-lymphoma-the-zeno-study
#6
LETTER
Ginevra Lolli, Lisa Argnani, Alessandro Broccoli, Miriam Marangon, Cinzia Pellegrini, Alice Morigi, Beatrice Casadei, Laura Nanni, Vittorio Stefoni, Matteo Carella, Michele Cavo, Pier L Zinzani
No abstract text is available yet for this article.
January 13, 2020: British Journal of Haematology
https://read.qxmd.com/read/31943138/addendum-to-british-society-for-haematology-guidelines-on-investigation-and-management-of-antiphospholipid-syndrome-2012-br-j-haematol-2012-157-47-58-use-of-direct-acting-oral-anticoagulants
#7
LETTER
Deepa R J Arachchillage, Keith Gomez, Raza Alikhan, Julia A M Anderson, Will Lester, Mike Laffan
No abstract text is available yet for this article.
January 13, 2020: British Journal of Haematology
https://read.qxmd.com/read/31943136/safety-and-efficacy-of-eltrombopag-plus-pulsed-dexamethasone-as-first-line-therapy-for-immune-thrombocytopenia
#8
Lunqing Zhang, Mingjie Zhang, Xin Du, Yunfeng Cheng, Gregory Cheng
Current first-line treatments for immune thrombocytopenia (ITP) usually have transient effects and sustained platelet response off therapy remains low. We evaluated whether eltrombopag plus pulsed dexamethasone as first-line therapy can increase the proportion of patients maintaining platelet counts >50 × 109 /l for a prolonged period without further ITP therapy. Treatment consisted of eltrombopag 25-75 mg daily according to platelet response for 12 weeks plus dexamethasone, 40 mg daily for four consecutive days every four weeks for 1-3 courses...
January 13, 2020: British Journal of Haematology
https://read.qxmd.com/read/31930481/a-major-break-of-cord-blood-bag
#9
LETTER
Takuji Matsuo, Jun Ooi, Haruko Tashiro, Sumiko Saito, Naoki Shirafuji
No abstract text is available yet for this article.
January 13, 2020: British Journal of Haematology
https://read.qxmd.com/read/31930492/the-meninges-enhance-leukaemia-survival-in-cerebral-spinal-fluid
#10
Patrick Basile, Leslie M Jonart, Maryam Ebadi, Kimberly Johnson, Morgan Kerfeld, Peter M Gordon
Central nervous system (CNS) relapse is a common cause of treatment failure in patients with acute lymphoblastic leukaemia (ALL) despite current CNS-directed therapies that are also associated with significant short- and long-term toxicities. Herein, we showed that leukaemia cells exhibit decreased proliferation, elevated reactive oxygen species (ROS) and increased cell death in cerebral spinal fluid (CSF) both in vitro and in vivo. However, interactions between leukaemia and meningeal cells mitigated these adverse effects...
January 12, 2020: British Journal of Haematology
https://read.qxmd.com/read/31900937/long-term-sustained-response-to-fostamatinib-in-two-patients-with-chronic-refractory-immune-thrombocytopenia-itp
#11
Eun-Ju Lee, Marina Izak, James B Bussel
Care of patients with chronic immune thrombocytopenia (ITP) who are refractory to available treatments can be quite challenging. Fostamatinib, an oral Syk inhibitor, is the newest FDA-approved agent for ITP. Phase 3 clinical trials demonstrated an overall response in 43% of patients treated with fostamatinib and use for two years has been reported. Herein, we report two patients with long histories of ITP without lasting responses to numerous first-, second- and third-line therapies with prolonged responses to ongoing fostamatinib...
January 3, 2020: British Journal of Haematology
https://read.qxmd.com/read/31900934/factor-viii-the-protein-cloning-its-gene-synthetic-factor-and-now-35-years-later-gene-therapy-what-happened-in-between
#12
REVIEW
Gavin Ling, Edward G D Tuddenham
The foundation of haemophilia A therapy in the last 35 years has been critically dependent on isolation of the Factor VIII (FVIII) protein and discovery of the cDNA sequence of the FVIII gene, published in 1984. Identification of the FVIII sequence resulted in a new era of recombinant concentrates and led to significant improvements in safety, set against the tragedy of widespread HIV and hepatitis infections in haemophilia patients from contaminated plasma-based products. We chronicle the scientific methods and race leading up to the publication of the FVIII DNA sequence and the legacy that follows through to revolutionary gene therapy treatment in clinical trials today...
January 3, 2020: British Journal of Haematology
https://read.qxmd.com/read/31900929/in-trans-early-mosaic-mutational-escape-and-novel-phenotypic-features-of-germline-samd9-mutation
#13
LETTER
Catherine Hockings, Satyen Gohil, Robin Dowse, Yvette Hoade, Marc R Mansour, Rosemary E Gale, David C Linch, Anupama Rao, Elspeth M Payne
No abstract text is available yet for this article.
January 3, 2020: British Journal of Haematology
https://read.qxmd.com/read/31898319/bortezomib-cyclophosphamide-dexamethasone-versus-lenalidomide-cyclophosphamide-dexamethasone-in-multiple-myeloma-patients-at-first-relapse
#14
Vittorio Montefusco, Alessandro Corso, Monica Galli, Ilaria Ardoino, Sara Pezzatti, Cristina Carniti, Francesca Patriarca, Filippo Gherlinzoni, Renato Zambello, Simona Sammassimo, Magda Marcatti, Andrea Nozza, Claudia Crippa, Anna Maria Cafro, Luca Baldini, Paolo Corradini
Bortezomib- and lenalidomide-containing regimens are well-established therapies in multiple myeloma (MM). However, despite their extensive use, head-to-head comparisons have never been performed. Therefore, we compared bortezomib and lenalidomide in fixed-duration therapies. In this open-label, phase III study, we randomized MM patients at first relapse to receive either nine cycles of bortezomib plus cyclophosphamide plus dexamethasone (VCD) or lenalidomide plus cyclophosphamide plus dexamethasone (RCD). The primary endpoint was achievement of a very good partial response (VGPR) or better at six weeks after nine treatment cycles...
January 2, 2020: British Journal of Haematology
https://read.qxmd.com/read/31889311/5-hydroxymethyl-furfural-restores-low-oxygen-rheology-of-sickle-trait-blood-in-vitro
#15
Scott Hansen, David K Wood, John M Higgins
Sickle cell trait (SCT) is the benign heterozygous carrier state for the sickle variant of the HBB gene. Most of the ~300 million people with SCT worldwide will not experience any significant complications. However, accumulating evidence finds SCT associated with increased risk for the common conditions of chronic kidney disease and venous thromboembolism, and severe but rare renal medullary carcinoma and exercise-induced rhabdomyolysis. The mechanism is uncertain, but probably involves pathological rheology of SCT blood in regions of low oxygen tension, resulting from sickle haemoglobin polymerization in SCT red cells and leading to reduced blood flow and further tissue hypoxia and damage...
December 30, 2019: British Journal of Haematology
https://read.qxmd.com/read/31885080/mucormycosis-in-children-with-haematological-malignancies-is-a-salvageable-disease-a-report-from-the-israeli-study-group-of-childhood-leukemia
#16
Sarah Elitzur, Nira Arad-Cohen, Assaf Barg, Naomi Litichever, Bella Bielorai, Ronit Elhasid, Salvador Fischer, Yariv Fruchtman, Gil Gilad, Joseph Kapelushnik, Mira Kharit, Osnat Konen, Ruth Laor, Itzhak Levy, Dror Raviv, Yael Shachor-Meyouhas, Yulia Shvartser-Beryozkin, Amos Toren, Isaac Yaniv, Ronit Nirel, Shai Izraeli, Shlomit Barzilai-Birenboim
Mucormycosis has emerged as an increasingly important cause of morbidity and mortality in immunocompromised patients, but contemporary data in children are lacking. We conducted a nationwide multicentre study to investigate the characteristics of mucormycosis in children with haematological malignancies. The cohort included 39 children with mucormycosis: 25 of 1136 children (incidence 2·2%) with acute leukaemias prospectively enrolled in a centralized clinical registry in 2004-2017, and an additional 14 children with haematological malignancies identified by retrospective search of the databases of seven paediatric haematology centres...
December 29, 2019: British Journal of Haematology
https://read.qxmd.com/read/31884679/increased-ferritin-levels-in-non-transfusion-dependent-%C3%AE-%C3%A2-thalassaemia-hbe-are-associated-with-reduced-cxcr2-expression-and-neutrophil-migration
#17
Chayada Thiengtavor, Sirikwan Siriworadetkun, Kittiphong Paiboonsukwong, Suthat Fucharoen, Kovit Pattanapanyasat, Jim Vadolas, Saovaros Svasti, Pornthip Chaichompoo
Severe bacterial infection is a major complication causing morbidity and mortality in β-thalassaemia/HbE patients. Innate immunity constitutes the first line of defence against bacterial infection. This study aimed to comprehensively investigate the innate immune phenotype and function related to factors predisposing to infection in non-transfusion-dependent (NTD) β°-thalassaemia/HbE patients. Twenty-six patients and 17 healthy subjects were recruited to determine complement activity (C3, C4, mannose-binding lectin and CH50) and surface receptor expression including markers of phagocytosis (CD11b, CD16 and C3bR), inflammation (C5aR) and migration (CD11b, CXCR1 and CXCR2) on neutrophils and monocytes...
December 29, 2019: British Journal of Haematology
https://read.qxmd.com/read/31883112/acute-lymphoblastic-leukaemia-patients-treated-with-pegasparaginase-develop-antibodies-to-peg-and-the-succinate-linker
#18
Robin Kloos, Inge M van der Sluis, Enrico Mastrobattista, Wim Hennink, Rob Pieters, Jan-Jaap Verhoef
Polyethylene glycol (PEG) conjugated asparaginase (PEGasparaginase) is essential for treatment of paediatric acute lymphoblastic leukaemia. We developed an assay identifying antibodies against the PEG-moiety, the linker and the drug itself in patients experiencing hypersensitivity reactions to PEGasparaginase. Eighteen patients treated according to the DCOG ALL-11 protocol, with a neutralizing hypersensitivity reaction to PEGasparaginase to the first PEGasparaginase doses in induction (12 patients) or during intensification after interruption of several months (6 patients) were included...
December 27, 2019: British Journal of Haematology
https://read.qxmd.com/read/31880329/what-do-we-know-about-duodenal-type-follicular-lymphoma-from-pathological-definition-to-treatment-options
#19
REVIEW
Guilherme Duffles Amarante, Graham Collins, Vanderson Rocha
Duodenal-type follicular lymphoma (DFL) is a newly recognised variant of follicular lymphoma (FL), although little is known about its biology and clinical evolution. In general, patients tend to have mild symptoms and do not require therapy, comparable with other forms of low-tumour burden asymptomatic FL. Specific pathological features, such as a dendritic cell meshwork, low expression of CD10 and upregulation of activation-induced cytidine deaminase can help the diagnosis. The molecular landscape of DFL is similar to the classical nodal presentation of FL, although studies using gene expression profiling demonstrate a close relation with MALT lymphomas...
December 27, 2019: British Journal of Haematology
https://read.qxmd.com/read/31880320/mutational-features-of-myelodysplastic-syndromes-with-auer-rods-reveal-them-are-more-akin-to-acute-myeloid-leukemia
#20
LETTER
Huijun Huang, Tiejun Qin, Zefeng Xu, Zhongxun Shi, Bing Li, Lijuan Pan, Naibo Hu, Shiqiang Qu, Gang Huang, Robert P Gale, Zhijian Xiao
No abstract text is available yet for this article.
December 27, 2019: British Journal of Haematology
journal
journal
20057
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"