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American Journal of Hematology

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https://www.readbyqxmd.com/read/28913922/a-clinically-meaningful-fetal-hemoglobin-threshold-for-children-with-sickle-cell-anemia-during-hydroxyurea-therapy
#1
Jeremie H Estepp, Matthew P Smeltzer, Guolian Kang, Chen Li, Winfred C Wang, Christina Abrams, Banu Aygun, Russell E Ware, Kerri Nottage, Jane S Hankins
Hydroxyurea has proven clinical benefits and is recommended to be offered to all children with sickle cell anemia (SCA), but the optimal dosing regimen remains controversial. Induction of red blood cell fetal hemoglobin (HbF) by hydroxyurea appears to be dose-dependent. However, it is unknown whether maximizing HbF% improves clinical outcomes. HUSTLE (NCT00305175) is a prospective observational study with a primary goal of describing the long-term clinical effects of hydroxyurea escalated to maximal tolerated dose (MTD) in children with SCA...
September 14, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28891083/a-nationwide-survey-of-hypoplastic-myelodysplastic-syndrome-a-multicenter-retrospective-study
#2
Takashi Kobayashi, Yasuhito Nannya, Motoshi Ichikawa, Kenji Oritani, Yuzuru Kanakura, Akihiro Tomita, Hitoshi Kiyoi, Masayoshi Kobune, Junji Kato, Hiroshi Kawabata, Motohiro Shindo, Yoshihiro Torimoto, Yuji Yonemura, Nobuyoshi Hanaoka, Hideki Nakakuma, Daisuke Hasegawa, Atsushi Manabe, Naohito Fujishima, Nobuharu Fujii, Mitsune Tanimoto, Yasuyoshi Morita, Akira Matsuda, Atsushi Fujieda, Naoyuki Katayama, Haruhiko Ohashi, Hirokazu Nagai, Yoshiki Terada, Masayuki Hino, Ken Sato, Naoshi Obara, Shigeru Chiba, Kensuke Usuki, Masatsugu Ohta, Osamu Imataki, Makiko Uemura, Tomoiku Takaku, Norio Komatsu, Akira Kitanaka, Kazuya Shimoda, Kenichiro Watanabe, Kaoru Tohyama, Akifumi Takaori-Kondo, Hideo Harigae, Shunya Arai, Yasushi Miyazaki, Keiya Ozawa, Mineo Kurokawa
Hypoplastic myelodysplastic syndrome (hMDS) is a distinct entity with bone marrow (BM) hypocellularity and the risk of death from BM failure (BMF). To elucidate the characteristics of hMDS, the data of 129 patients diagnosed between April 2003 and March 2012 were collected from 20 institutions and the central review team of the National Research Group on Idiopathic Bone Marrow Failure Syndromes, and compared with 115 non-hMDS patients. More RA and fewer CMMoL and RAEB-t in French-American-British (FAB) and more RCUD and MDS-U and fewer RCMD in World Health Organization (WHO) classifications were found in hMDS than non-hMDS with significant differences...
September 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28891226/clinical-management-of-peripherally-inserted-central-catheters-piccs-compared-to-conventional-central-venous-catheters-ccvcs-in-patients-with-hematological-malignancies-a-large-multicenter-study-of-the-rel-group-rete-ematologica-lombarda-lombardy-hematologic
#3
LETTER
Nicola Stefano Fracchiolla, Elisabetta Todisco, Andrea Bilancia, Sara Gandolfi, Nicola Orofino, Francesca Guidotti, Valentina Mancini, Laura Marbello, Andrea Assanelli, Massimo Bernardi, Armando Santoro, Roberto Cairoli, Dario Consonni, Agostino Cortelezzi
No abstract text is available yet for this article.
September 10, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28891213/identification-of-novel-mutations-in-the-hbf-repressor-gene-bcl11a-in-patients-with-autism-and-intelligence-disabilities
#4
LETTER
Tao Cai, Xiang Chen, Jinchen Li, Bingwu Xiang, Liu Yang, Yidian Liu, Qiuli Chen, Zhouwen He, Kevin Sun, P Paul Liu
No abstract text is available yet for this article.
September 10, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28875545/targeted-next-generation-sequencing-in-myelodysplastic-syndromes-and-prognostic-interaction-between-mutations-and-ipss-r
#5
Ayalew Tefferi, Terra L Lasho, Mrinal M Patnaik, Lyla Saeed, Mythri Mudireddy, Dame Idossa, Christy Finke, Rhett P Ketterling, Animesh Pardanani, Naseema Gangat
A 27-gene panel was used for next-generation sequencing (NGS) in 179 patients (median age 73 years) with primary myelodysplastic syndromes (MDS); risk distribution according to the revised International Prognostic Scoring System (IPSS-R) was 11% very high, 18% high, 17% intermediate, 38% low and 16% very low. At least one mutation/variant was detected in 147 (82%) patients; 23% harbored three or more mutations/variants. The most frequent mutations/variants included ASXL1 (30%), TET2 (25%), SF3B1 (20%), U2AF1 (16%), SRSF2 (16%), TP53 (13%), RUNX1 (11%) and DNMT3A (10%)...
September 5, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28857304/causes-of-hospital-admission-in-%C3%AE-thalassemia-chat-in-lebanon-from-1995-to-2015-a-pilot-retrospective-study-from-a-tertiary-care-center
#6
LETTER
Antoine N Saliba, Hassan M Moukhadder, Afif Harb, Hassan Beydoun, Rayan Bou-Fakhredin, Ali T Taher
No abstract text is available yet for this article.
August 31, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28857249/enhancing-diversity-in-the-hematology-biomedical-research-workforce-a-mentoring-program-to-improve-the-odds-of-career-success-for-early-stage-investigators
#7
Betty S Pace, Levi H Makala, Rita Sarkar, Li Liu, Mayuko Takezaki, Narla Mohandas, Glorias Dixon, Ellen M Werner, Donna B Jeffe, Treva K Rice, Nita J Maihle, Juan González
No abstract text is available yet for this article.
August 31, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28850704/long-term-survival-of-beta-thalassemia-major-patients-treated-with-hematopoietic-stem-cell-transplantation-compared-with-survival-with-conventional-treatment
#8
Giovanni Caocci, Maria Grazia Orofino, Adriana Vacca, Antonio Piroddi, Eugenia Piras, Maria Carmen Addari, Rossella Caria, Maria Paola Pilia, Raffaella Origa, Paolo Moi, Giorgio La Nasa
Allogeneic hematopoietic stem cell transplantation (HSCT) in thalassemia remains a challenge. We reported a single-centre case-control study of a large cohort of 516 children and adult patients treated with HSCT or blood transfusion support and iron chelation therapy; 258 patients (median age 12, range 1-45) underwent sibling (67%) or unrelated (33%) HSCT; 97 patients were adults (age ≥ 16 years). The median follow-up after HSCT was 11 years (range 1-30). The conditioning regimen was busulfan (80.6%) or treosulfan-based (19...
August 29, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28850699/clinical-outcomes-in-adult-patients-with-aplastic-anemia-a-single-institution-experience
#9
Prajwal Boddu, Guillermo Garcia-Manero, Farhad Ravandi, Gautam Borthakur, Elias Jabbour, Courtney DiNardo, Nitin Jain, Naval Daver, Naveen Pemmaraju, Paolo Anderlini, Simrit Parmar, Devendra Kc, Mary Akosile, Sherry A Pierce, Richard Champlin, Jorge Cortes, Hagop Kantarjian, Tapan Kadia
Newer treatment modalities are being investigated to improve upon historical outcomes with standard immunosuppressive therapy (IST) in aplastic anemia (AA). We analyzed outcomes of adult patients with AA sequentially treated with various combinatorial anti-thymoglobulin-based IST regimens in frontline and relapsed/refractory (R/R) settings. Pretreatment and on-treatment clinical characteristics were analyzed for relationships to response and outcome. Among 126 patients reviewed, 95 were treatment-naïve (TN) and 63, R/R (including 32 from the TN cohort); median ages were 49 and 50 years, respectively...
August 29, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28842936/a-single-center-phase-ii-study-of-ixazomib-in-patients-with-relapsed-or-refractory-cutaneous-or-peripheral-t-cell-lymphomas
#10
Philip S Boonstra, Avery Polk, Noah Brown, Alexandra C Hristov, Nathanael G Bailey, Mark S Kaminski, Tycel Phillips, Sumana Devata, Tera Mayer, Ryan A Wilcox
The transcription factor GATA-3, highly expressed in many cutaneous and peripheral T-cell lymphomas, confers resistance to chemotherapy in a cell-autonomous manner. As GATA-3 is transcriptionally regulated by NF-κB, we sought to determine the extent to which proteasomal inhibition impairs NF-κB activation and GATA-3 expression and cell viability in malignant T cells. Proteasome inhibition, NF-κB activity, GATA-3 expression, and cell viability were examined in patient-derived cell lines and primary T-cell lymphoma specimens ex vivo treated with the oral proteasome inhibitor ixazomib...
August 26, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28842931/changes-in-intestinal-microbiota-and-their-effects-on-allogeneic-stem-cell-transplantation
#11
REVIEW
Rory M Shallis, Christopher M Terry, Seah H Lim
The human intestinal microbiota is essential for microbial homeostasis, regulation of metabolism, and intestinal immune tolerance. Rapidly evolving understanding of the importance of the microbiota implicates changes in the composition and function of intestinal microbial communities in an assortment of systemic conditions. Complications following allogeneic stem cell transplant now join the ever-expanding list of pathologic states regulated by intestinal microbiota. Dysbiosis, or disruption of the normal ecology of this microbiome, has been directly implicated in the pathogenesis of entities such as Clostridium difficile infections, graft-versus-host disease, and most recently disease relapse, all of which are major causes of morbidity and mortality in patients undergoing allogeneic stem cell transplant...
August 26, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28836284/imatinib-induced-psoriasiform-eruption-in-a-patient-with-chronic-myeloid-leukemia
#12
Connie R Shi, Vinod E Nambudiri
No abstract text is available yet for this article.
August 24, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28833466/clinical-and-laboratory-findings-associated-with-sleep-disordered-breathing-in-sickle-cell-disease
#13
LETTER
Christopher M Worsham, Stephon T Martin, Mehdi Nouraie, Robyn T Cohen, Elizabeth S Klings
No abstract text is available yet for this article.
August 22, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28833417/comprehensive-evaluation-of-the-revised-international-staging-system-in-multiple-myeloma-patients-treated-with-novel-agents-as-a-primary-therapy
#14
Hyungwoo Cho, Dok Hyun Yoon, Jung Bok Lee, Sung-Yong Kim, Joon Ho Moon, Young Rok Do, Jae Hoon Lee, Yong Park, Ho Sup Lee, Hyeon Seok Eom, Ho-Jin Shin, Chang-Ki Min, Jin Seok Kim, Jae-Cheol Jo, Hye Jin Kang, Yeung-Chul Mun, Won Sik Lee, Je-Jung Lee, Cheolwon Suh, Kihyun Kim
The revised International Staging System (R-ISS) has recently been developed to improve the risk stratification of multiple myeloma (MM) patients over the ISS. We assessed the R-ISS in MM patients who were treated with novel agents as a primary therapy and evaluated its discriminative power and ability to reclassify patients from the ISS. A total of 514 newly diagnosed MM patients treated with novel agents including thalidomide, bortezomib, and lenalidomide as a primary therapy were included in this retrospective analysis...
August 22, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28833370/considerations-on-poems-associated-optic-neuropathy
#15
LETTER
Julio González Martín-Moro, Inés Contreras Martín, María Castro-Rebollo, Belén Pilo de la Fuente
No abstract text is available yet for this article.
August 22, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28815757/first-line-treatment-selection-and-early-monitoring-patterns-in-chronic-phase-chronic-myeloid-leukemia-in-routine-clinical-practice-simplicity
#16
Stuart L Goldberg, Jorge Cortes, Carlo Gambacorti-Passerini, Rüdiger Hehlmann, H Jean Khoury, Mauricette Michallet, Ron Paquette, Bengt Simonsson, Teresa Zyczynski, Aimee Foreman, Elisabetta Abruzzese, David Andorsky, Aart Beeker, Pascale Cony-Makhoul, Richard Hansen, Elza Lomaia, Eduardo Olavarria, Michael Mauro
Achieving successful outcomes in chronic phase-chronic myeloid leukemia (CP-CML) requires careful monitoring of cytogenetic/molecular responses (CyR/MR). SIMPLICITY (NCT01244750) is an observational study exploring tyrosine kinase inhibitor use and management patterns in patients with CP-CML receiving first-line imatinib (n=416), dasatinib (n=418) or nilotinib (n=408) in the US and 6 European countries in routine clinical practice. Twelve-month follow-up data of 1,242 prospective patients (enrolled October 01 2010-September 02 2015) are reported...
August 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28815695/a-distinctive-histidine-residue-is-essential-for-in-vivo-glycation-inactivation-of-human-cd59-transgenically-expressed-in-mice-erythrocytes-implications-for-human-diabetes-complications
#17
Rupam Sahoo, Pamela Ghosh, Michael Chorev, Jose A Halperin
Clinical and experimental evidences support a link between the complement system and the pathogenesis of diabetes complications. CD59, an extracellular cell membrane-anchored protein, inhibits formation of the membrane attack complex (MAC), the main effector of complement-mediated tissue damage. This complement regulatory activity of human CD59 (hCD59) is inhibited by hyperglycemia-induced ε-amino glycation of Lys(41) . Biochemical and structural analyses of glycated proteins with known three-dimensional structure revealed that glycation of ε-amino lysyl residues occurs predominantly at "glycation motives" that include lysyl/lysyl pairs or proximity of a histidyl residue, in which the imidazolyl moiety is ≈ 5Å from the ε-amino group...
August 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28815693/ibrutinib-associated-pneumocystis-jirovecii-pneumonia
#18
LETTER
Regina Lee, Afrouz Nayernama, S Christopher Jones, Tanya Wroblewski, Peter E Waldron
No abstract text is available yet for this article.
August 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28815690/treatment-of-individuals-who-cannot-receive-blood-products-for-religious-or-other-reasons
#19
REVIEW
Carlton D Scharman, Joseph J Shatzel, Edward Kim, Thomas G DeLoughery
By virtue of their religious principles, Jehovah's Witnesses (JWs) generally object to receiving blood products, raising numerous ethical, legal, and medical challenges for providers who care for these patients, especially in the emergent setting. In this review, we discuss several areas relevant to the care of JWs, including the current literature on "bloodless" medical care in the setting of peri- and intra-operative management, acute blood loss, trauma, pregnancy, and malignancy. We have found that medical and administrative efforts in the form of bloodless medicine and surgery programs can be instrumental in helping to reduce risks of morbidity and mortality in these patients...
August 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28815688/bmp2-controls-iron-homeostasis-in-mice-independent-of-bmp6
#20
Susanna Canali, Chia-Yu Wang, Kimberly B Zumbrennen-Bullough, Abraham Bayer, Jodie L Babitt
Hepcidin is a key iron regulatory hormone that controls expression of the iron exporter ferroportin to increase the iron supply when needed to support erythropoiesis and other essential functions, but to prevent the toxicity of iron excess. The bone morphogenetic protein (BMP)-SMAD signaling pathway, through the ligand BMP6 and the co-receptor hemojuvelin, is a central regulator of hepcidin transcription in the liver in response to iron. Here, we show that dietary iron loading has a residual ability to induce Smad signaling and hepcidin expression in Bmp6-/- mice, effects that are blocked by a neutralizing BMP2/4 antibody...
August 17, 2017: American Journal of Hematology
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