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Bone Marrow Failure

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https://www.readbyqxmd.com/read/28530668/radioimmunotherapy-augmented-beam-chemotherapy-vs-beam-alone-as-the-high-dose-regimen-for-autologous-stem-cell-transplantation-asct-in-relapsed-follicular-lymphoma-fl-a-retrospective-study-of-the-ebmt-lymphoma-working-party
#1
L Bento, A Boumendil, H Finel, S Le Gouill, S Amorim, H Monjanel, R Bouabdallah, J O Bay, E Nicolas-Virelizier, G McQuaker, G Rossi, R Johnson, A Huynh, P Ceballos, A Rambaldi, E Bachy, R Malladi, K Orchard, D Pohlreich, H Tilly, F Bonifazi, X Poiré, F Guilhot, A Haenel, C Crawley, B Metzner, J Gribben, N H Russell, G Damaj, K Thomson, P Dreger, S Montoto
Relapse remains the most common cause of treatment failure in patients receiving autologous stem cell transplantation (ASCT) for follicular lymphoma (FL). The aim of this study was to evaluate the effect of adding radioimmunotherapy or rituximab (R) to BEAM (carmustine, etoposide, ara-c, melphalan) high-dose therapy for ASCT in patients with relapsed FL. Using the European Society for Blood and Marrow Transplantation registry, we conducted a cohort comparison of BEAM (n=1973), Zevalin-BEAM (Z-BEAM) (n=207) and R-BEAM (n=179) and also a matched-cohort analysis of BEAM vs Z-BEAM including 282 and 154 patients, respectively...
May 22, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28530458/-early-symptoms-of-childhood-malignant-diseases
#2
Zsuzsanna Erzsébet Papp, Izabella Kelemen, Adrienne Horváth
INTRODUCTION: Childhood malignant diseases are rare in pediatric pathology. Early symptoms are not specific, fatigue, pallor, compression signs and bone marrow failure are often mentioned. AIM: To summarize the most frequent early symptoms of childhood malignancies in order to help the physicians in the early recognition. METHOD: In our retrospective study, we processed a period of 5 years between 2012-2016, with an accent on the onset manifestations of malignancies...
May 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28523571/congenital-immature-pure-erythroid-leukemia-with-e-cadherin-expression
#3
Akihiro Tamura, Suguru Uemura, Atsuro Saito, Saki Okubo, Nanako Nino, Teppei Tahara, Takehito Yokoi, Kenji Kishimoto, Toshiaki Ishida, Daiichiro Hasegawa, Keiichiro Kawasaki, Seiji Yoshimoto, Hideto Nakao, Makiko Yoshida, Yoshiyuki Kosaka
Congenital pure erythroid leukemia is exceedingly rare and poses a diagnostic challenge. We report an atypical case of congenital pure erythroid leukemia that did not express typical erythroid markers. The patient presented with a high white blood cell count with blastic cells at birth. Although flow cytometric analyses of peripheral blood and bone marrow showed a large CD45-negative cell population, we did not identify any evidence of monoclonality. While the circulating blasts decreased with only supportive care, hepatomegaly with multiple nodules was accompanied by liver failure, disseminated intravascular coagulation, and development of hemophagocytic lymphohistiocytosis...
May 18, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28522358/self-tolerance-and-autoimmunity-in-a-minimal-model-of-the-idiotypic-network
#4
Stefan Landmann, Nicolas Preuss, Ulrich Behn
We consider self-tolerance and its failure -autoimmunity- in a minimal mathematical model of the idiotypic network. A node in the network represents a clone of B-lymphocytes and its antibodies of the same idiotype which is encoded by a bitstring. The links between nodes represent possible interactions between clones of almost complementary idiotype. A clone survives only if the number of populated neighbored nodes is neither too small nor too large. The dynamics is driven by the influx of lymphocytes with randomly generated idiotype from the bone marrow...
May 15, 2017: Journal of Theoretical Biology
https://www.readbyqxmd.com/read/28516949/paroxysmal-nocturnal-haemoglobinuria
#5
REVIEW
Anita Hill, Amy E DeZern, Taroh Kinoshita, Robert A Brodsky
Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal haematopoietic stem cell (HSC) disease that presents with haemolytic anaemia, thrombosis and smooth muscle dystonias, as well as bone marrow failure in some cases. PNH is caused by somatic mutations in PIGA (which encodes phosphatidylinositol N-acetylglucosaminyltransferase subunit A) in one or more HSC clones. The gene product of PIGA is required for the biosynthesis of glycosylphosphatidylinositol (GPI) anchors; thus, PIGA mutations lead to a deficiency of GPI-anchored proteins, such as complement decay-accelerating factor (also known as CD55) and CD59 glycoprotein (CD59), which are both complement inhibitors...
May 18, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28512561/bone-marrow-homing-and-engraftment-defects-of-human-hematopoietic-stem-and-progenitor-cells
#6
REVIEW
Giovanni Caocci, Marianna Greco, Giorgio La Nasa
Homing of hematopoietic stem cells (HSC) to their microenvironment niches in the bone marrow is a complex process with a critical role in repopulation of the bone marrow after transplantation. This active process allows for migration of HSC from peripheral blood and their successful anchoring in bone marrow before proliferation. The process of engraftment starts with the onset of proliferation and must, therefore, be functionally dissociated from the former process. In this overview, we analyze the characteristics of stem cells (SCs) with particular emphasis on their plasticity and ability to find their way home to the bone marrow...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28512217/loss-of-the-homologous-recombination-gene-rad51-leads-to-fanconi-anemia-like-symptoms-in-zebrafish
#7
Jan Gregor Botthof, Ewa Bielczyk-Maczyńska, Lauren Ferreira, Ana Cvejic
RAD51 is an indispensable homologous recombination protein, necessary for strand invasion and crossing over. It has recently been designated as a Fanconi anemia (FA) gene, following the discovery of two patients carrying dominant-negative mutations. FA is a hereditary DNA-repair disorder characterized by various congenital abnormalities, progressive bone marrow failure, and cancer predisposition. In this report, we describe a viable vertebrate model of RAD51 loss. Zebrafish rad51 loss-of-function mutants developed key features of FA, including hypocellular kidney marrow, sensitivity to cross-linking agents, and decreased size...
May 16, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28507545/clinical-and-molecular-heterogeneity-of-rtel1-deficiency
#8
Carsten Speckmann, Sushree Sangita Sahoo, Marta Rizzi, Shinsuke Hirabayashi, Axel Karow, Nina Kathrin Serwas, Marc Hoemberg, Natalja Damatova, Detlev Schindler, Jean-Baptiste Vannier, Simon J Boulton, Ulrich Pannicke, Gudrun Göhring, Kathrin Thomay, J J Verdu-Amoros, Holger Hauch, Wilhelm Woessmann, Gabriele Escherich, Eckart Laack, Liliana Rindle, Maximilian Seidl, Anne Rensing-Ehl, Ekkehart Lausch, Christine Jandrasits, Brigitte Strahm, Klaus Schwarz, Stephan R Ehl, Charlotte Niemeyer, Kaan Boztug, Marcin W Wlodarski
Typical features of dyskeratosis congenita (DC) resulting from excessive telomere shortening include bone marrow failure (BMF), mucosal fragility, and pulmonary or liver fibrosis. In more severe cases, immune deficiency and recurring infections can add to disease severity. RTEL1 deficiency has recently been described as a major genetic etiology, but the molecular basis and clinical consequences of RTEL1-associated DC are incompletely characterized. We report our observations in a cohort of six patients: five with novel biallelic RTEL1 mutations p...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28505185/combined-treatment-of-miltefosine-and-paromomycin-delays-the-onset-of-experimental-drug-resistance-in-leishmania-infantum
#9
Sarah Hendrickx, Magali Van den Kerkhof, Dorien Mabille, Paul Cos, Peter Delputte, Louis Maes, Guy Caljon
BACKGROUND: Since miltefosine monotherapy against visceral leishmaniasis (VL) caused by Leishmania donovani has been discontinued in the Indian subcontinent due to an increase in the number of treatment failures, single dose liposomal amphotericin B is now advocated as a treatment option of choice. Paromomycin-miltefosine combination therapy can be used as substitute first-line treatment in regions without cold-chain potential. Previous laboratory studies in the closely related species Leishmania infantum have demonstrated that paromomycin monotherapy fairly rapidly selects for resistance producing a phenotype with increased fitness...
May 15, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28500307/alox5-exhibits-anti-tumor-and-drug-sensitizing-effects-in-mll-rearranged-leukemia
#10
Yungui Wang, Jennifer R Skibbe, Chao Hu, Lei Dong, Kyle Ferchen, Rui Su, Chenying Li, Hao Huang, Hengyou Weng, Huilin Huang, Xi Qin, Jie Jin, Jianjun Chen, Xi Jiang
MLL-rearranged acute myeloid leukemia (AML) remains a fatal disease with a high rate of relapse and therapeutic failure due to chemotherapy resistance. In analysis of our Affymetrix microarray profiling and chromatin immunoprecipitation (ChIP) assays, we found that ALOX5 is especially down-regulated in MLL-rearranged AML, via transcription repression mediated by Polycomb repressive complex 2 (PRC2). Colony forming/replating and bone marrow transplantation (BMT) assays showed that Alox5 exhibited a moderate anti-tumor effect both in vitro and in vivo...
May 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28497239/validation-of-the-revised-international-prognostic-scoring-system-in-patients-with-myelodysplastic-syndrome-in-japan-results-from-a-prospective-multicenter-registry
#11
Hiroshi Kawabata, Kaoru Tohyama, Akira Matsuda, Kayano Araseki, Tomoko Hata, Takahiro Suzuki, Hidekazu Kayano, Kei Shimbo, Yuji Zaike, Kensuke Usuki, Shigeru Chiba, Takayuki Ishikawa, Nobuyoshi Arima, Masaharu Nogawa, Akiko Ohta, Yasushi Miyazaki, Kinuko Mitani, Keiya Ozawa, Shunya Arai, Mineo Kurokawa, Akifumi Takaori-Kondo
The Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes has been conducting prospective registration, central review, and follow-up study for patients with aplastic anemia and myelodysplastic syndrome (MDS) since 2006. Using this database, we retrospectively analyzed the prognosis of patients with MDS. As of May 2016, 351 cases were registered in this database, 186 of which were eligible for the present study. Kaplan-Meier analysis showed that overall survival (OS) curves of the five risk categories stipulated by the revised international prognostic scoring system (IPSS-R) were reasonably separated...
May 11, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28495927/an-infant-with-pearson-syndrome-a-rare-cause-of-congenital-sideroblastic-anemia-and-bone-marrow-failure
#12
Corey P Falcon, Thomas H Howard
No abstract text is available yet for this article.
May 11, 2017: Blood
https://www.readbyqxmd.com/read/28494518/correlates-of-resistance-and-relapse-during-blinatumomab-therapy-for-relapsed-refractory-acute-lymphoblastic-leukemia
#13
Ibrahim Aldoss, Joo Song, Tracey Stiller, Tina Nguyen, Joycelynne Palmer, Margaret O'Donnell, Anthony S Stein, Guido Marcucci, Stephen Forman, Vinod Pullarkat
We retrospectively analyzed 65 patients with refractory/relapsed (r/r) ALL who were treated with blinatumomab for predictors of leukemia response as well as clinical patterns of relapse and resistance with particular focus on downregulation of CD19 expression and extramedullary disease (EM-ALL). The complete remission (CR) rate was 51%, and 15 (45%) responders underwent allogeneic hematopoietic cell transplantation (HCT) in CR. High leukemia burden (bone marrow blasts >50%) (P=0.02), history of prior EM-ALL (P=0...
May 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28493328/screening-of-181-patients-with-antibody-deficiency-for-deficiency-of-adenosine-deaminase-2-sheds-new-light-on-the-disease-in-adulthood
#14
Johanna Schepp, Michele Proietti, Natalie Frede, Mary Buchta, Katrin Hübscher, Jessica Rojas Restrepo, Sigune Goldacker, Klaus Warnatz, Jana Pachlopnik Schmid, Andrea Duppenthaler, Vassilios Lougaris, Ignacio Uriarte, Susan Kelly, Michael Hershfield, Bodo Grimbacher
OBJECTIVE: We aimed to test the relevance of Deficiency of Adenosine Deaminase 2 in patients with antibody deficiency and describe the clinical picture of the disease in adulthood. METHODS: We screened for DADA2 in a cohort of 181 patients with antibody deficiency with and without vascular lesions by the means of Next-Generation-Sequencing and targeted Sanger Sequencing. All mutations were confirmed by determining the ADA2 enzymatic activity levels in dried plasma spots...
May 10, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28493158/common-variable-immunodeficiency-caused-by-fanc-mutations
#15
Yujin Sekinaka, Noriko Mitsuiki, Kohsuke Imai, Miharu Yabe, Hiromasa Yabe, Kanako Mitsui-Sekinaka, Kenichi Honma, Masatoshi Takagi, Ayako Arai, Kenichi Yoshida, Yusuke Okuno, Yuichi Shiraishi, Kenichi Chiba, Hiroko Tanaka, Satoru Miyano, Hideki Muramatsu, Seiji Kojima, Asuka Hira, Minoru Takata, Osamu Ohara, Seishi Ogawa, Tomohiro Morio, Shigeaki Nonoyama
Common variable immunodeficiency (CVID) is the most common adult-onset primary antibody deficiency disease due to various causative genes. Several genes, which are known to be the cause of different diseases, have recently been reported as the cause of CVID in patients by performing whole exome sequencing (WES) analysis. Here, we found FANC gene mutations as a cause of adult-onset CVID in two patients. B cells were absent and CD4(+) T cells were skewed toward CD45RO(+) memory T cells. T-cell receptor excision circles (TRECs) and signal joint kappa-deleting recombination excision circles (sjKRECs) were undetectable in both patients...
May 11, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28491865/the-role-of-the-central-nervous-system-microenvironment-in-pediatric-acute-lymphoblastic-leukemia
#16
REVIEW
Nathan P Gossai, Peter M Gordon
Acute lymphoblastic leukemia (ALL) is the most common cancer in children. While survival rates for ALL have improved, central nervous system (CNS) relapse remains a significant cause of treatment failure and treatment-related morbidity. Accordingly, there is a need to identify more efficacious and less toxic CNS-directed leukemia therapies. Extensive research has demonstrated a critical role of the bone marrow (BM) microenvironment in leukemia development, maintenance, and chemoresistance. Moreover, therapies to disrupt mechanisms of BM microenvironment-mediated leukemia survival and chemoresistance represent new, promising approaches to cancer therapy...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28489771/chronic-active-epstein-barr-virus-infection-associated-with-hemophagocytic-syndrome-and-extra-nodal-natural-killer-t-cell-lymphoma-in-an-18-year-old-girl-a-case-report
#17
Yawei Xing, Junwen Yang, Guanghui Lian, Shuijiao Chen, Linlin Chen, Fujun Li
RATIONALE: Chronic active Epstein-Barr virus infection (CAEBV) associated with hemophagocytic syndrome (HPS) and extra-nodal natural killer (NK)/T-cell lymphoma (ENKL) is a rare life-threatening disorder. This disease is easily misdiagnosed because of its varied presentations. PATIENT CONCERNS: An 18-year-old girl was admitted to our hospital with a history of edema in the lower limbs and intermittent fever lasting for more than 1 month. At admission, she had severe liver injury of unknown etiology...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28487294/the-effect-of-variation-in-donor-platelet-function-on-transfusion-outcome-a-semi-randomised-controlled-trial
#18
Anne M Kelly, Stephen F Garner, Theodora Foukaneli, Thomas R Godec, Nina Herbert, Brennan C Kahan, Alison Deary, Lekha Bakrania, Charlotte Llewelyn, Willem H Ouwehand, Lorna M Williamson, Rebecca A Cardigan
The effect of variation in platelet function in platelet donors on patient outcome following platelet transfusion is unknown. This trial assessed the hypothesis that platelets collected from donors with highly responsive platelets to agonists in vitro assessed by flow cytometry (high responder donors), are cleared more quickly from the circulation than those from low responder donors, resulting in lower platelet count increments following transfusion. This parallel group, semi-randomised double-blinded trial was conducted in a single UK centre...
May 9, 2017: Blood
https://www.readbyqxmd.com/read/28486441/bone-mineral-density-in-patients-with-inherited-bone-marrow-failure-syndromes
#19
Roopa Kanakatti Shankar, Neelam Giri, Maya B Lodish, Ninet Sinaii, James C Reynolds, Sharon A Savage, Constantine A Stratakis, Blanche P Alter
BACKGROUNDPatients with inherited bone marrow failure syndromes (IBMFS) may have several risk factors for low bone mineral density (BMD). We aimed to evaluate the prevalence of low BMD in IBMFS and determine associated risk factors.METHODSPatients with IBMFS with at least one Dual Energy X-ray absorptiometry (DXA) scan were evaluated. Diagnosis of each IBMFS, Fanconi anemia (FA), dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome, was confirmed by syndrome-specific tests. Data were gathered on age, height and clinical history...
May 9, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28485976/use-of-nanoparticle-contrast-agents-for-cell-tracking-with-computed-tomography
#20
Johoon Kim, Peter Chhour, Jessica Hsu, Harold I Litt, Victor A Ferrari, Rachela Popovtzer, David P Cormode
Efforts to develop novel cell-based therapies originated with the first bone marrow transplant on a leukemia patient in 1956. Preclinical and clinical examples of cell-based treatment strategies have shown promising results across many disciplines in medicine, with recent advances in immune cell therapies for cancer producing remarkable response rates, even in patients with multiple treatment failures. However, cell-based therapies suffer from inconsistent outcomes, motivating the search for tools that allow monitoring cell delivery and behavior in vivo...
May 9, 2017: Bioconjugate Chemistry
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