journal
https://read.qxmd.com/read/38696714/tlr7-signalling-a-central-nexus-in-autoimmunity-and-cgvhd
#1
JOURNAL ARTICLE
Ranjeet Singh Mahla
No abstract text is available yet for this article.
May 2, 2024: Blood Advances
https://read.qxmd.com/read/38696711/cardiovascular-toxicity-of-bruton-tyrosine-kinase-inhibitors-forget-about-selectivity-but-watch-the-clock
#2
JOURNAL ARTICLE
Giorgio Minotti
No abstract text is available yet for this article.
May 2, 2024: Blood Advances
https://read.qxmd.com/read/38696707/the-clinical-trials-landscape-in-immunoglobulin-light-chain-amyloidosis-a-systematic-review
#3
JOURNAL ARTICLE
Ramya Ramachandran, Darshi Shah, Catherine Luo, Veer Shah, Edward Robert Scheffer Cliff, Vaishali Sanchorawala, Suzanne Lentzsch, Rajshekhar Chakraborty
No abstract text is available yet for this article.
May 2, 2024: Blood Advances
https://read.qxmd.com/read/38695879/ash-guideline-oversight-subcommittee-response-to-jacobs-et-al-industry-payments-to-ash-guideline-panelists
#4
JOURNAL ARTICLE
Menaka Pai, Michael T Byrne, Adam Cuker, Benjamin Djulbegovic, James N George, Matthew D Seftel, Deirdra R Terrell, Robert Kunkle, Matthew C Cheung
No abstract text is available yet for this article.
May 2, 2024: Blood Advances
https://read.qxmd.com/read/38691583/cytokine-profiling-in-128-patients-with-transient-abnormal-myelopoiesis-a-report-from-the-jplsg-tam-10-trial
#5
JOURNAL ARTICLE
Genki Yamato, Yusuke Tsumura, Hideki Muramatsu, Akira Shimada, Takahiro Imaizumi, Hiroyuki Tsukagoshi, Taeko Kaburagi, Norio Shiba, Yoshiyuki Yamada, Takao Deguchi, Tomoko Kawai, Kiminori Terui, Etsuro Ito, Kenichiro Watanabe, Yasuhide Hayashi
Transient abnormal myelopoiesis (TAM) occurs in 10% of neonates with Down syndrome (DS). Although most patients show spontaneous resolution of TAM, early death occurs in approximately 20% of cases. Therefore, new biomarkers are needed to predict early death and determine therapeutic interventions. This study aimed to determine the association between clinical characteristics and cytokine levels in patients with TAM. A total of 128 patients with DS with TAM enrolled in the TAM-10 study conducted by the Japanese Pediatric Leukemia/Lymphoma Study Group were included in this study...
May 1, 2024: Blood Advances
https://read.qxmd.com/read/38687368/long-term-outcomes-of-peripheral-blood-stem-cell-unrelated-donors-mobilized-with-filgrastim
#6
JOURNAL ARTICLE
Heather E Stefanski, Michelle Kuxhausen, Stephanie Bo-Subait, Hati Kobusingye, Deborah Mattila, Jennifer Schenfeld, Darcie Sandschafer, Linda J Burns, Bronwen E Shaw, Michael A Pulsipher, John P Miller, Steven M Devine
Allogeneic hematopoietic cell transplantation is a life-saving procedure used to treat of a variety of devastating diseases. It requires hematopoietic stem cells collected via filgrastim mobilized peripheral blood stem cells or bone marrow harvest from volunteer unrelated donors. There is a paucity of safety data regarding donors' long-term adverse events. This prospective, observational study combined peripheral blood stem cell donors enrolled on the NMDP Investigational New Drug trial and bone marrow donors between July 1, 1999, and September 30, 2015...
April 30, 2024: Blood Advances
https://read.qxmd.com/read/38669353/defining-primary-refractory-large-b-cell-lymphoma
#7
JOURNAL ARTICLE
Allison M Bock, Raphael Mwangi, Yucai Wang, Arushi Khurana, Matthew J Maurer, Amy Ayers, Brad S Kahl, Peter Martin, Jonathon B Cohen, Carla Casulo, Izidore S Lossos, Umar Farooq, Sabarish Ayyappan, Tanner Wayne Reicks, Thomas M Habermann, Thomas E Witzig, Christopher R Flowers, James R Cerhan, Loretta J Nastoupil, Grzegorz S Nowakowski
Patients with large B-cell lymphoma (LBCL) that fail to achieve a complete response (CR) or relapse early after anthracycline-containing immunochemotherapy (IC) have a poor prognosis and are commonly considered "primary refractory disease". However, different definitions of primary refractory disease are used in the literature and clinical practice. In this study, we ex-amined variation in the time to relapse used to define refractory status and association with sur-vival outcomes in patients with primary refractory LBCL in a single-center prospective cohort with a validation in an independent multi-center cohort...
April 26, 2024: Blood Advances
https://read.qxmd.com/read/38669351/venous-thromboembolism-characteristics-and-outcomes-among-riete-patients-tested-untested-for-inherited-thrombophilia
#8
JOURNAL ARTICLE
Omri Cohen, Liat Waldman Radinsky, Gili Kenet, Isabelle Mahe, Giovanni Barillari, Silvia Soler, Patricia Sigüenza, Maria Del Valle Morales, Paula Villares, Manuel Monreal
Inherited thrombophilia (IT) workup is commonly pursued in venous thromboembolism (VTE) patients. Recent American Society of Hematology (ASH) guidelines recommend a selective approach to IT testing, nevertheless, evidence on whether thrombophilia testing can actually improve patient-important outcomes through tailored management is limited. Data from the large prospective RIETE registry was analyzed to compare VTE risk factors, management and outcomes between patients who were tested for IT and untested patients, during anticoagulant treatment and following its discontinuation...
April 26, 2024: Blood Advances
https://read.qxmd.com/read/38669350/displace-study-shows-poor-quality-of-transcranial-doppler-ultrasound-for-stroke-risk-screening-in-sickle-cell-anemia
#9
JOURNAL ARTICLE
Kimberly Ann Davidow, Robin Miller, Shannon M Phillips, Alyssa M Schlenz, Martina Mueller, Monica L Hulbert, Lewis L Hsu, Neha Bhasin, Robert J Adams, Julie Kanter
Children with sickle cell anemia (SCA) are at increased risk of stroke when compared to age-based counterparts. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) previously demonstrated that with the use of transcranial Doppler ultrasound (TCD; Sickle Stroke Screen) and chronic red cell transfusion, the risk of stroke risk is reduced by over 90%. The STOP criteria detailed the type and method of measurement required; the time averaged mean maximum velocity (TAMMV). Unfortunately, it has been difficult to adhere to the appropriate TAMMV measurements...
April 26, 2024: Blood Advances
https://read.qxmd.com/read/38669344/myosin-1c-augments-secretion-of-von-willebrand-factor-by-linking-contractile-actomyosin-machinery-to-the-plasma-membrane
#10
JOURNAL ARTICLE
Sammy El-Mansi, Tom P Mitchell, Golzar Mobyen, Thomas Aj McKinnon, Pika Miklavc, Manfred Frick, Thomas D Nightingale
Blood endothelial cells control the hemostatic and inflammatory response by secreting von Willebrand factor (VWF) and P-selectin from storage organelles called Weibel-Palade bodies (WPB). Actin-associated motor proteins regulate this secretory pathway at multiple points. Prior to fusion, myosin Va forms a complex that anchors WPBs to peripheral actin structures allowing maturation of content. Post-fusion, an actomyosin ring/coat is recruited and compresses the WPB to forcibly expel the largest VWF multimers...
April 26, 2024: Blood Advances
https://read.qxmd.com/read/38669341/modified-delphi-panel-consensus-recommendations-for-management-of-severe-aplastic-anemia
#11
JOURNAL ARTICLE
Daria V Babushok, Amy E DeZern, Carlos de Castro, Zora R Rogers, David Beenhouwer, Michael S Broder, Suzanne Fanning, Sarah N Gibbs, Rabi Hanna, Jaroslaw P Maciejewski, Bart L Scott, Srinivas K Tantravahi, Marcin W Wlodarski, Irina Yermilov, Bhumika J Patel
Severe aplastic anemia (SAA) is a rare hematologic condition for which there is no clear management algorithm. A panel of 11 adult and pediatric experts on aplastic anemia was assembled and, using the RAND/UCLA modified Delphi panel method, evaluated >600 varying patient care scenarios to develop clinical recommendations for the initial and subsequent management of patients of all ages with SAA. Here we present the panel's recommendations to rule out inherited bone marrow failure (IBMF) syndromes, on supportive care prior to and during first-line therapy, and on first-line (initial management) and second-line (subsequent management) therapy of acquired SAA, focusing on when transplant versus medical therapy is most appropriate...
April 26, 2024: Blood Advances
https://read.qxmd.com/read/38669315/high-dose-alemtuzumab-cyclosporine-vs-tacrolimus-methotrexate-sirolimus-for-chronic-graft-versus-host-disease-prevention
#12
JOURNAL ARTICLE
Noa G Holtzman, Lauren M Curtis, Rachel B Salit, Brian C Shaffer, Filip Pirsl, Alen Ostojic, Seth M Steinberg, Eduard Schulz, Jennifer S Wilder, Thomas E Hughes, Jeremy Rose, Sarfraz Memon, Robert Korngold, Juan Gea-Banacloche, Daniel H Fowler, Frances T Hakim, Ronald E Gress, Michael R Bishop, Steven Z Pavletic
Chronic graft-versus-host disease (cGVHD) remains a significant problem for patients after allogeneic hematopoietic stem cell transplants (allo-HSCT). While in vivo lymphodepletion by antibodies for cGVHD prophylaxis has been explored in the myeloablative setting, its effects after reduced intensity conditioning (RIC) are not well described. Patients (n=83) with hematologic malignancies underwent targeted lymphodepletion chemotherapy followed by a RIC allo-HSCT using peripheral blood stem cells from unrelated donors...
April 26, 2024: Blood Advances
https://read.qxmd.com/read/38662646/maternal-red-blood-cell-alloimmunization-prevalence-in-the-united-states
#13
JOURNAL ARTICLE
Ronan P Sugrue, Kenneth J Moise, Jerome J Federspiel, Elizabeth Abels, Judy Z Louie, Zhen Chen, Lance A Bare, Damian P Alagia, Harvey W Kaufman
Hemolytic disease of fetus and newborn (HDFN) is a life-threatening disease mediated by maternal alloimmunization to red blood cell (RBC) antigens. Studies of maternal alloimmunization prevalence in the United States (U.S.) lack national data. This study describes prevalence and trends in alloimmunization in pregnancy in the U.S. RBC antibodies (abs) were identified in a large, nationwide, commercial laboratory database from 2010-2021. The cohort comprised pregnancies for which the year of lab collection and patient's state of residence were available...
April 25, 2024: Blood Advances
https://read.qxmd.com/read/38662645/don-t-keep-me-waiting-estimating-the-lifetime-impact-of-reduced-vein-to-vein-time-for-car-t-treated-patients-with-lbcl
#14
JOURNAL ARTICLE
Sachin Vadgama, Marcelo C Pasquini, Richard T Maziarz, Zhen-Huan Hu, Markqayne D Ray, Harry Smith, Ash Bullement, Mark Edmondson-Jones, Will Sullivan, Guillaume Cartron
Chimeric antigen receptor (CAR) T-cell therapies have revolutionized the treatment of hematological cancers. Production requires a complex logistical process from leukapheresis to patient infusion, the vein-to-vein time (V2VT), during which a patients clinical condition may deteriorate. This study was designed to estimate the benefits of reduced V2VT for third-line+ (3L+) relapsed/refractory large B-cell lymphoma (r/r LBCL) patients treated with CAR T. A mathematical model was developed to estimate the lifetime outcomes of a hypothetical cohort of patients who had either a 'long' or 'short' V2VT...
April 25, 2024: Blood Advances
https://read.qxmd.com/read/38662475/unravelling-facets-of-mecom-associated-syndrome-somatic-genetic-rescue-clonal-hematopoiesis-and-phenotype-expansion
#15
JOURNAL ARTICLE
Parvathy Venugopal, Peer Arts, Lucy Claire Fox, Annet Simons, Devendra K Hiwase, Peter G Bardy, Annette Narcis, David M Ross, Lize F D van Vulpen, Arjan Buijs, Kelly L Bolton, Bartlomiej Getta, Eliska Furlong, Tina Carter, Ingrid Krapels, Marlijn P A Hoeks, Adila Al Kindy, Farah Al Kindy, Sonja de Munnik, Pamela Evans, Mahalia S B Frank, Adam Bournazos, Sandra T Cooper, Thuong Thi Ha, Matilda R Jackson, Luis Alberto Arriola-Martinez, Kerry Phillips, Yvonne Brennan, Madhura Bakshi, Karen Ambler, Song Gao, Karin S Kassahn, Rosalie Kenyon, Kevin Hung, Milena Babic, Alan McGovern, Lesley Rawlings, Cassandra Valkulin, Lucas Dejong, Rema Fathi, Simon McRae, Nicholas Myles, Dariusz Ladon, Marjolijn C Jongmans, Roland P Kuiper, Nicola Poplawski, Pasquale M Barbaro, Piers Blombery, Anna L Brown, Christopher N Hahn, Hamish S Scott
No abstract text is available yet for this article.
April 25, 2024: Blood Advances
https://read.qxmd.com/read/38661372/trends-in-volumes-and-survival-after-hematopoietic-cell-transplantation-in-racial-ethnic-minorities
#16
JOURNAL ARTICLE
Nandita Khera, Sikander Ailawadhi, Ruta Brazauskas, Jinalben Patel, Benjamin Jacobs, Celalettin Ustun, Karen Ballen, Muhammad Bilal Abid, Miguel A Díaz, A Samar Al-Homsi, Hasan Hashem, Sanghee Hong, Reinhold Munker, Raquel Schears, Hillard M Lazarus, Stefan O Ciurea, Sherif M Badawy, Bipin N Savani, Baldeep Wirk, Charles F LeMaistre, Neel S Bhatt, Amer Beitinjaneh, Mahmoud Aljurf, Akshay Sharma, Jan Cerny, Jennifer M Knight, Amar H Kelkar, Jean A Yared, Tamila Kindwall-Keller, Lena E Winestone, Amir Steinberg, Staci D Arnold, Sachiko Seo, Jaime M Preussler, Nasheed M Hossain, Warren B Fingrut, Vaibhav Agrawal, Shahrukh Hashmi, Leslie Elaine Lehmann, William A Wood, Hemalatha G Rangarajan, Wael Saber, Theresa Hahn
There has been an increase in volume as well as improvement in overall survival (OS) after hematopoietic cell transplantation (HCT) for hematologic disorders. It is unknown if these changes have impacted racial/ethnic minorities equally. In this observational study from Center for International Blood and Marrow Transplant Research of 79,904 autologous (auto) and 65,662 allogeneic (allo) HCTs, we examined the volume and rates of change of auto HCT and allo HCT over time and trends in OS in 4 racial/ethnic groups: Non-Hispanic Whites (NHWs), Non-Hispanic African Americans (NHAAs), Hispanics across five 2-year cohorts from 2009 to 2018...
April 25, 2024: Blood Advances
https://read.qxmd.com/read/38652890/clonal-hematopoiesis-of-indeterminate-potential-in-patients-with-immunoglobulin-light-chain-al-amyloidosis
#17
JOURNAL ARTICLE
Paolo Lopedote, Benjamin Evans, Alfredo Marchetti, Tianzeng Chen, Maria Moscvin, Samuel Boullt, Niccolo Bolli, Giada Bianchi
Immunoglobulin light chain (AL) amyloidosis is characterized by the deposition of misfolded monoclonal free light chains, with cardiac complications accounting for patients' mortality. Clonal hematopoiesis of indeterminate potential (CHIP) has been associated with worse cardiovascular outcomes in the general population. Its significance in AL amyloidosis remains unclear. We collected clinical information and outcome data on 76 patients with a diagnosis of AL amyloidosis who underwent deep-targeted sequencing for myeloid neoplasia-associated mutations between April 2018 and August 2023...
April 23, 2024: Blood Advances
https://read.qxmd.com/read/38640438/o-glycan-determinants-regulate-vwf-trafficking-to-weibel-palade-bodies
#18
JOURNAL ARTICLE
Ellie Karampini, Dearbhla Doherty, Petra E Bürgisser, Massimiliano Garre, Ingmar Schoen, Stephanie Elliott, Ruben Bierings, James S O'Donnell
Von Willebrand factor (VWF) undergoes complex post-translational modification within endothelial cells (EC) prior to secretion. This includes significant N- and O-linked glycosylation. Previous studies have demonstrated that changes in N-linked glycan structures significantly influence VWF biosynthesis. In contrast, although abnormalities in VWF O-linked glycans (OLG) have been associated with enhanced VWF clearance, their effect on VWF biosynthesis remains poorly explored. Herein, we report a novel role for OLG determinants in regulating VWF biosynthesis and trafficking within EC...
April 19, 2024: Blood Advances
https://read.qxmd.com/read/38640437/rates-of-severe-neutropenia-and-infection-risk-in-patients-treated-with-deferiprone-28-years-of-data
#19
JOURNAL ARTICLE
Sherif M Badawy, Jan Palmblad, Fernando Tricta, Noemi Toiber Temin, Caroline Fradette, Leo Lin, Anna Rozova, Sujit Sheth
Patients treated with deferiprone for transfusional iron overload may experience idiosyncratic drug-induced neutropenia (IDIN) that may put them at risk of infection. The purpose of this analysis was to examine the rates of severe IDIN and risk of serious infections at different ANC levels in patients treated with deferiprone. Events of severe IDIN (ANC <0.5×109/L) and associated serious infections from clinical trials and postmarketing setting were analyzed by 3 discrete ANC levels: Group 1, 0.2-0...
April 19, 2024: Blood Advances
https://read.qxmd.com/read/38640435/lysosomal-degradation-targets-mutant-calreticulin-and-the-thrombopoietin-receptor-in-myeloproliferative-neoplasms
#20
JOURNAL ARTICLE
Amanpreet Kaur, Arunkumar Venkatesan, Malathi Kandarpa, Moshe Talpaz, Malini Raghavan
Somatic mutants of calreticulin (CRT) drive myeloproliferative neoplasms (MPNs) via binding to the thrombopoietin receptor (MPL) and aberrant activation of the JAK/STAT pathway. Compared with healthy donors, platelets from MPN patients with CRT mutations display low cell surface MPL. Additionally, co-expression of MPL with an MPN-linked CRT mutant (CRTDel52) reduces cell surface MPL, suggesting that CRTDel52 may induce MPL degradation. We show that lysosomal degradation is relevant to the turnover of CRTDel52 and MPL...
April 19, 2024: Blood Advances
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