journal
https://read.qxmd.com/read/36971095/binge-reading-on-immune-thrombocytopenia-everything-you-ever-wanted-to-know
#1
JOURNAL ARTICLE
Rick Kapur, John W Semple
Immune thrombocytopenia (ITP) is a complex clinical and pathophysiological autoimmune disorder and in the past decade, thousands of papers have been published on this topic. To shed light on the global scientific output, Ou et al. performed a comprehensive bibliometric analysis of the ITP literature to clarify the major hotspots and future research directions. Commentary on: Ou et al. A bibliometric analysis of primary immune thrombocytopenia from 2011 to 2021. Br J Haematol 2023 (Online ahead of print). doi: 10...
March 27, 2023: British Journal of Haematology
https://read.qxmd.com/read/36971083/secondary-spleen-in-immune-thrombocytopenia-not-so-accessory-after-all%C3%A2
#2
JOURNAL ARTICLE
Rick Kapur, Sylvain Audia
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by an isolated thrombocytopenia. The pathophysiology is complex but involves platelet-autoantibodies and/or cytotoxic T cells, with the spleen playing an important regulatory role. Accessory spleen (AcS) may possibly contribute to ITP relapse following splenectomy; however, the microenvironment of AcS has not been directly compared to the main spleen. Pizzi et al. conducted a histological study of adult ITP patients where they compared eight matched AcS to main spleens, and they observed a similar immunological composition in both groups...
March 27, 2023: British Journal of Haematology
https://read.qxmd.com/read/36971077/occult-optic-nerve-neurolymphomatosis-of-t-lymphoblastic-lymphoma-detected-by-pet-mri
#3
JOURNAL ARTICLE
Kai Shen, Chenlu Yang
No abstract text is available yet for this article.
March 27, 2023: British Journal of Haematology
https://read.qxmd.com/read/36971072/the-continuing-global-challenges-of-treating-patients-with-beta-thalassemia
#4
JOURNAL ARTICLE
Ariel Koren
In this issue, Hokland et al. offer an interesting view of the different approaches on how to treat a beta-thalassemia patient. The principal concerns, that this report reveals, are the very wide differences in the facilities and economic resources available for the care of patients. Management of thalassemia should become a word wide health care priority and may include at last those two steps: national and international registries; national programs for screening couples at risk and providing preventive measures to prevent births of patients with thalassemia...
March 27, 2023: British Journal of Haematology
https://read.qxmd.com/read/36971071/temporary-contraindication-to-chemotherapy-due-to-toxicity-blinatumomab-s-effectiveness-in-paediatric-patients-with-b-acute-lymphoblastic-leukaemia
#5
LETTER
C Collignon, C Domenech, S Ducassou, C Pluchart, B Bruno, M Pasquet, P Simon, A Petit, F Rialland-Battisti, B Brethon
No abstract text is available yet for this article.
March 27, 2023: British Journal of Haematology
https://read.qxmd.com/read/36971063/the-oldest-case-of-lymphoma-insights-from-a-xiii-century-fresco
#6
JOURNAL ARTICLE
Francesco Lanza, Roberto De Giorgio, Giorgio Zavagli
No abstract text is available yet for this article.
March 27, 2023: British Journal of Haematology
https://read.qxmd.com/read/36971061/idelalisib-plus-rituximab-versus-ibrutinib-in-the-treatment-of-relapsed-refractory-chronic-lymphocytic-leukaemia-a-real-world-analysis-from-the-chronic-lymphocytic-leukemia-patients-registry-cllear
#7
JOURNAL ARTICLE
Martin Špaček, Lukáš Smolej, Martin Šimkovič, Lucie Nekvindová, Zlatuše Křístková, Yvona Brychtová, Anna Panovská, Stanislava Mašlejová, Lucie Bezděková, Dominika Écsiová, Pavel Vodárek, Jana Zuchnická, Jana Mihályová, Renata Urbanová, Peter Turcsányi, Daniel Lysák, Jan Novák, Martin Brejcha, Tereza Líkařová, Prokop Vodička, Jana Baranová, Marek Trněný, Michael Doubek
Idelalisib (idela), a phosphatidylinositol 3-kinase inhibitor, and ibrutinib, a Bruton tyrosine kinase inhibitor, were the first oral targeted agents approved for relapsed/refractory (R/R) chronic lymphocytic leukaemia (CLL). However, no randomised trials of idelalisib plus rituximab (R-idela) versus ibrutinib have been conducted. Therefore, we performed a real-world retrospective analysis of patients with R/R CLL treated with R-idela (n = 171) or ibrutinib (n = 244). The median age was 70 versus 69 years, with a median of two previous lines...
March 27, 2023: British Journal of Haematology
https://read.qxmd.com/read/36965115/acute-chest-syndrome-in-adult-patients-with-sickle-cell-disease-the-relationship-with-the-time-to-onset-after-hospital-admission
#8
JOURNAL ARTICLE
Geoffrey Cheminet, Antoine Brunetti, Djamal Khimoud, Brigitte Ranque, Adrien Michon, Edouard Flamarion, Jacques Pouchot, Anne-Sophie Jannot, Jean-Benoît Arlet
Data on acute chest syndrome (ACS) in adult sickle cell disease patients are scarce. In this study, we describe 105 consecutive ACS episodes in 81 adult patients during a 32-month period and compare the characteristics as a function of the time to onset after hospital admission for a vaso-occlusive crisis (VOC), that is early-onset episodes (time to onset ≤24 h, 42%) versus secondary episodes (>24 h, 58%; median [interquartile range] time to onset: 2 [2-3] days). The median age was 27 [22-34] years, 89% of the patients had an S/S or S/β0 -thalassaemia genotype; 81% of the patients had a history of ACS (median: 3 [2-5] per patient), only 61% were taking a disease-modifying treatment at the time of the ACS...
March 25, 2023: British Journal of Haematology
https://read.qxmd.com/read/36965050/pathophysiology-of-gastrointestinal-acute-graft-versus-host-disease-and-the-potential-role-of-glucagon-like-peptide-2
#9
REVIEW
Robert Zeiser, Yi-Bin Chen, Nader N Youssef, Francis Ayuk
Acute graft-versus-host disease (aGVHD) is a life-threatening complication after allogeneic haematopoietic cell transplantation, with gastrointestinal (GI) tract involvement (GI aGVHD) being one of the leading causes of morbidity and mortality. Whilst systemic steroids are the standard first-line treatment for aGVHD, approximately 50% of patients become steroid refractory (SR), which is associated with poor outcomes. Existing options for SR-GVHD are limited, and there is a significant unmet need for new non-immunosuppressive treatment approaches in patients with GI aGVHD...
March 25, 2023: British Journal of Haematology
https://read.qxmd.com/read/36964994/hyperammonemia-and-acute-liver-failure-associated-with-deferasirox-in-two-adolescents-with-sickle-cell-disease
#10
LETTER
Alison S Towerman, Kristin P Guilliams, Réjean Guerriero, Marwan S Shinawi, Janis M Stoll, Daniel N Willis, Monica L Hulbert
No abstract text is available yet for this article.
March 25, 2023: British Journal of Haematology
https://read.qxmd.com/read/36960712/gbt021601-improves-red-blood-cell-health-and-the-pathophysiology-of-sickle-cell-disease-in-a-murine-model
#11
JOURNAL ARTICLE
Kobina Dufu, Carsten Alt, Steven Strutt, James Partridge, Tzechiang Tang, Vincent Siu, Hilary Liao-Zou, Peter Rademacher, Alexander T Williams, Cynthia R Muller, Xin Geng, Mira Patel Pochron, Annie Nguyen Dang, Pedro Cabrales, Zhe Li, Donna Oksenberg, Brian E Cathers
The pathophysiologic mechanism of sickle cell disease (SCD) involves polymerization of deoxygenated haemoglobin S (HbS), leading to red blood cell (RBC) sickling, decreased RBC deformability, microvascular obstruction, haemolysis, anaemia and downstream clinical complications. Pharmacological increase in the concentration of oxygenated HbS in RBCs has been shown to be a novel approach to inhibit HbS polymerization and reduce RBC sickling and haemolysis. We report that GBT021601, a small molecule that increases HbS-oxygen affinity, inhibits HbS polymerization and prevents RBC sickling in blood from patients with SCD...
March 24, 2023: British Journal of Haematology
https://read.qxmd.com/read/36951891/extensive-histiocytosis-masking-anaplastic-large-cell-lymphoma-alcl
#12
JOURNAL ARTICLE
Shauna Wentzell, Barbara Morash, Hong Chang
No abstract text is available yet for this article.
March 23, 2023: British Journal of Haematology
https://read.qxmd.com/read/36951360/structural-and-functional-characterization-of-novel-f7-mutations-identified-in-chinese-factor-vii-deficient-patients
#13
JOURNAL ARTICLE
Can Lou, Jiali Jiang, Weizhi Chen, Zhili Zhang, Guanqun Xu, Yu Liu, Jing Dai, Qiulan Ding, Xuefeng Wang, Hongying Wei, Youwei Wu, Qin Xu, Wenman Wu
Hereditary factor VII (FVII) deficiency is a rare recessive bleeding disorder with an estimated prevalence of 1/500 000. We had investigated 50 unrelated Chinese patients with FVII deficiency and identified, in total, 25 mutations, including 18 missense mutations and 5 splicing mutations, on the F7 gene. The nucleotide transition c.1224T>G (p.His408Gln) in exon 9 constitutes a hotspot of mutation, with 19 patients harbouring this genetic variance. Few patients were homozygous or compound heterozygous for deleterious mutations, such as non-sense mutations, large insertion or deletions, indicating that complete deficiency of FVII may not be compatible with life...
March 23, 2023: British Journal of Haematology
https://read.qxmd.com/read/36951293/outcomes-of-patients-with-therapy-related-myeloid-neoplasms-after-treatment-with-poly-adp-ribose-polymerase-proteins-inhibitors-for-solid-tumours
#14
LETTER
Emmanuel Almanza-Huante, Alex Bataller, Samuel Urrutia, Georgina Gener-Ricos, Robert Edward Briski, Rashmi Kanagal-Shamanna, Karen H Lu, Shannon N Westin, Timothy A Yap, Koichi Takahashi, Farhad Ravandi, Yesid Alvarado, Tapan Kadia, Koiji Sasaki, Hagop M Kantarjian, Guillermo Garcia-Manero
No abstract text is available yet for this article.
March 23, 2023: British Journal of Haematology
https://read.qxmd.com/read/36951278/venetoclax-ramp-up-strategies-for-chronic-lymphocytic-leukaemia-in-the-united-kingdom-a-real-world-multicentre-retrospective-study
#15
JOURNAL ARTICLE
Rocio Figueroa-Mora, Alexandros Rampotas, Daniel Halperin, Tina Worth, Jennifer Vidler, Dario Melotti, Paul Ferguson, Nagah Elmusharaf, Gavin Preston, Michelle Furtado, Moez Dungarwalla, Satyen Gohill, Piers Patten, Ben Kennedy, Toby A Eyre, Anna Schuh, Christopher P Fox, Tahla Munir, Nicolas Martinez-Calle
This retrospective, observational study evaluated patterns of inpatient versus outpatient tumour lysis syndrome (TLS) monitoring during venetoclax ramp-up in 170 patients with chronic lymphocytic leukaemia. The primary outcome was clinical/biochemical TLS. Two clinical and four biochemical TLS occurred (4.1%). Five of the six events occurred in high-risk patients, four occurred at 20 mg dose and three at the 6-h time-point. Inpatient versus outpatient TLS rates within the high-risk subgroup were 15% and 8%...
March 23, 2023: British Journal of Haematology
https://read.qxmd.com/read/36951259/a-mirna-signature-related-to-stemness-identifies-high-risk-patients-in-paediatric-acute-myeloid-leukaemia
#16
JOURNAL ARTICLE
Elena Esperanza-Cebollada, Soledad Gómez-González, Sara Perez-Jaume, Nerea Vega-García, Clara Vicente-Garcés, Mercè Richarte-Franqués, Susana Rives, Albert Català, Montserrat Torrebadell, Mireia Camós
Clinical and biological variables like genetic aberrations at diagnosis and the levels of measurable residual disease (MRD) are the most powerful biomarkers to predict the outcome of paediatric leukaemia. Recently, a model integrating the genetic abnormalities, transcriptional identity, and leukaemia stemness measured as leukaemic stem cell score (pLSC6) has been proposed to identify high-risk paediatric acute myeloid leukaemia (AML) patients. However, the role of epigenetics in defining prognosis still needs to be established...
March 23, 2023: British Journal of Haematology
https://read.qxmd.com/read/36949658/impact-of-measurable-residual-disease-on-outcomes-of-unrelated-donor-haematopoietic-cell-transplantation-with-post-transplant-cyclophosphamide-in-aml-in-first-complete-remission
#17
JOURNAL ARTICLE
Arnon Nagler, Myriam Labopin, Bhagirathbhai Dholaria, Didier Blaise, Sergey Bondarenko, Jan Vydra, Goda Choi, Montserrat Rovira, Péter Reményi, Ellen Meijer, Claude Eric Bulabois, J L Diez-Martin, Ibrahim Yakoub-Agha, Eolia Brissot, Alexandros Spyridonidis, Jaime Sanz, Amit Patel, Mutlu Arat, Ali Bazarbachi, Gesine Bug, Bipin N Savani, Sebastian Giebel, Fabio Ciceri, Mohamad Mohty
Pre-transplant measurable residual disease (MRD) predicts relapse and outcome of allogeneic haematopoietic cell transplantation (allo-HCT). The impact of MRD on the outcomes of post-transplant cyclophosphamide (PTCy)-based allo-HCT from a matched unrelated donor (UD) is unknown. This study assessed the impact of MRD in acute myeloid leukaemia (AML) in the first complete remission (CR1). A total of 272 patients (MRD negative [MRD-], n = 165; MRD positive [MRD+], n = 107) with a median follow-up of 19 (range: 16-24) months were studied...
March 22, 2023: British Journal of Haematology
https://read.qxmd.com/read/36949601/donor-type-red-blood-cell-transfusion-to-deplete-isoagglutinins-prior-to-allogeneic-stem-cell-transplantation-from-abo-major-incompatible-bone-marrow-donors
#18
JOURNAL ARTICLE
Andrea Jarisch, Emilia Salzmann-Manrique, Jan Soerensen, Gudrun Sach, Eva Rettinger, Andre Willasch, Shahrzad Bakhtiar, Dieter Klarmann, Susanne Bräuninger, Laura Moser, Julia Fekadu, Martin Hutter, Thomas Klingebiel, Jan-Henning Klusmann, Peter Bader, Halvard Bonig
ABO incompatibility affects approximately 40% of allogeneic stem cell transplants in Caucasian patient populations. Because bone marrow (BM), the preferred graft from paediatric sibling donors and for non-malignant diseases, has a red blood cell (RBC) content similar to blood, anti-donor isoagglutinins must either be depleted from the recipient or RBCs removed from the graft. To achieve tolerability of unmanipulated BM grafts, we used controlled infusions of donor ABO-type RBC units to deplete isoagglutinins before the transplant...
March 22, 2023: British Journal of Haematology
https://read.qxmd.com/read/36946218/post-transplant-lymphoproliferative-disorder-update-on-treatment-and-novel-therapies
#19
REVIEW
Suheil Albert Atallah-Yunes, Omar Salman, Michael J Robertson
Post-transplant lymphoproliferative disorder (PTLD) is rare and heterogeneous lymphoid proliferations that occur as a result of immunosuppression following solid organ transplant (SOT) and haematopoietic stem cell transplant (HSCT) with the majority being driven by EBV. Although some histologies are similar to lymphoid neoplasms seen in immunocompetent patients, treatment of PTLD may be different due to difference in pathobiology and higher risk of treatment complications. The most common treatment approach in SOT PTLD after failing immunosuppression reduction (RIS) takes into consideration a risk-stratified sequential algorithm with rituximab +/- chemotherapy based on phase 2 studies...
March 22, 2023: British Journal of Haematology
https://read.qxmd.com/read/36942786/burden-of-covid-19-disease-and-vaccine-coverages-in-apulian-splenectomized-patients-a-retrospective-observational-study
#20
JOURNAL ARTICLE
Francesco Paolo Bianchi, Pasquale Stefanizzi, Donato Rizzi, Noemi Signorile, Eustachio Cuscianna, Antonio Daleno, Giovanni Migliore, Silvio Tafuri
Splenectomy/asplenia is a condition associated with immune-compromission and specific vaccines are recommended for these patients, including the anti-COVID-19 vaccine. Among the high-risk group for which vaccination was prioritized in Italy, the immunocompromised patients after therapies or treatments were included. The Apulian regional archive of hospital discharge forms was used to define the list of splenectomized Apulian inhabitants, considering data from 2015 through 2020. The overall vaccination status of asplenic patients was assessed via data collected from the Regional Immunization Database...
March 21, 2023: British Journal of Haematology
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