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Myelodysplasia

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https://www.readbyqxmd.com/read/28095364/acute-myeloid-leukemia-with-myelodysplasia-related-changes-demonstrating-mixed-lineage-phenotype
#1
Babita Kajal, Hong Chang
No abstract text is available yet for this article.
September 22, 2016: Blood
https://www.readbyqxmd.com/read/28092887/acute-myeloid-leukemia-with-myelodysplasia-related-changes-demonstrating-prominent-basophilic-differentiation
#2
Mohammad Bahmanyar, Hong Chang
No abstract text is available yet for this article.
May 19, 2016: Blood
https://www.readbyqxmd.com/read/28090659/the-effect-of-intradetrusor-botulinum-neurotoxin-type-a-on-urinary-ngf-tgf-beta-1-timp-2-levels-in-children-with-neurogenic-detrusor-overactivity-due-to-myelodysplasia
#3
Tuncay Top, Cagri Akin Sekerci, Banu Isbilen-Basok, Yiloren Tanidir, Ilker Tinay, Ferruh Kemal Isman, Cem Akbal, Ferruh Simsek, Tufan Tarcan
AIMS: The aim of this study was to determine the value of urine nerve growth factor (NGF), transforming growth factor beta 1 (TGF-Beta-1), tissue inhibitor of matrix metalloproteinase 2 (TIMP-2) levels to predict the urodynamic profile before and after botulinum neurotoxin type A (BoNT-A) treatment in children with myelodysplasia. METHODS: This prospective study included 15 children with myelodysplasia who underwent intradetrusor BoNT-A injections due to neurogenic detrusor overactivity (NDOA)...
January 16, 2017: Neurourology and Urodynamics
https://www.readbyqxmd.com/read/28090092/the-clonal-origins-of-leukemic-progression-of-myelodysplasia
#4
T H Kim, M S Tyndel, H J Kim, J-S Ahn, S H Choi, H J Park, Y-K Kim, D-H Yang, J-J Lee, S-H Jung, S Y Kim, Y H Min, J-W Cheong, S K Sohn, J H Moon, M Choi, M Lee, Z Zhang, D Dong Hwan Kim
The genetics behind the progression of myelodysplasia to secondary acute myeloid leukemia (sAML) is poorly understood. In this study, we profiled somatic mutations and their dynamics using next generation sequencing on serial samples from a total of 124 patients, consisting of a 31 patient discovery cohort and 93 patients from two validation cohorts. Whole-exome analysis on the discovery cohort revealed that 29 of 31 patients carry mutations related to at least one of 8 commonly mutated pathways in AML. Mutations in genes related to DNA methylation and splicing machinery were found in T-cell samples, which expand at the initial diagnosis of the myelodysplasia, suggesting their importance as early disease events...
January 16, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28062854/sf3b1-mutations-associated-with-myelodysplastic-syndromes-alter-the-fidelity-of-branchsite-selection-in-yeast
#5
Tucker J Carrocci, Douglas M Zoerner, Joshua C Paulson, Aaron A Hoskins
RNA and protein components of the spliceosome work together to identify the 5' splice site, the 3' splice site, and the branchsite (BS) of nascent pre-mRNA. SF3b1 plays a key role in recruiting the U2 snRNP to the BS. Mutations in human SF3b1 have been linked to many diseases such as myelodysplasia (MDS) and cancer. We have used SF3b1 mutations associated with MDS to interrogate the role of the yeast ortholog, Hsh155, in BS selection and splicing. These alleles change how the spliceosome recognizes the BS and alter splicing when nonconsensus nucleotides are present at the -2, -1 and +1 positions relative to the branchpoint adenosine...
January 6, 2017: Nucleic Acids Research
https://www.readbyqxmd.com/read/28035072/%C3%AE-np73-overexpression-promotes-resistance-to-apoptosis-but-does-not-cooperate-with-pml-rara-in-the-induction-of-an-apl-leukemic-phenotype
#6
Antonio R Lucena-Araujo, Juan L Coelho-Silva, Diego A Pereira-Martins, Carolina Thomé, Priscila S Scheucher, Ana P Lange, Helder H Paiva, Benjamin T Hemmelgarn, Mariana C Morais-Sobral, Elisa A Azevedo, Pedro L Franca-Neto, Rafael F Franca, Cleide L Silva, Alexandre Krause, Eduardo M Rego
Here, we evaluated whether the overexpression of transcriptionally inactive ΔNp73 cooperates with PML/RARA fusion protein in the induction of an APL-leukemic phenotype, as well as its role in vitro in proliferation, myeloid differentiation, and drug-induced apoptosis. Using lentiviral gene transfer, we showed in vitro that ΔNp73 overexpression resulted in increased proliferation in murine bone marrow (BM) cells from hCG-PML/RARA transgenic mice and their wild-type (WT) counterpart, with no accumulation of cells at G2/M or S phases; instead, ΔNp73-expressing cells had a lower rate of induced apoptosis...
December 27, 2016: Oncotarget
https://www.readbyqxmd.com/read/28024152/ubiquitination-of-hnrnpa1-by-traf6-links-chronic-innate-immune-signaling-with-myelodysplasia
#7
Jing Fang, Lyndsey C Bolanos, Kwangmin Choi, Xiaona Liu, Susanne Christie, Shailaja Akunuru, Rupali Kumar, Dehua Wang, Xiaoting Chen, Kenneth D Greis, Peter Stoilov, Marie-Dominique Filippi, Jaroslaw P Maciejewski, Guillermo Garcia-Manero, Matthew T Weirauch, Nathan Salomonis, Hartmut Geiger, Yi Zheng, Daniel T Starczynowski
Toll-like receptor (TLR) activation contributes to premalignant hematologic conditions, such as myelodysplastic syndromes (MDS). TRAF6, a TLR effector with ubiquitin (Ub) ligase activity, is overexpressed in MDS hematopoietic stem/progenitor cells (HSPCs). We found that TRAF6 overexpression in mouse HSPC results in impaired hematopoiesis and bone marrow failure. Using a global Ub screen, we identified hnRNPA1, an RNA-binding protein and auxiliary splicing factor, as a substrate of TRAF6. TRAF6 ubiquitination of hnRNPA1 regulated alternative splicing of Arhgap1, which resulted in activation of the GTP-binding Rho family protein Cdc42 and accounted for hematopoietic defects in TRAF6-expressing HSPCs...
February 2017: Nature Immunology
https://www.readbyqxmd.com/read/27938958/anterior-ischemic-optic-neuropathy-and-hematologic-malignancy-a-systematic-review-of-case-reports-and-case-series
#8
David Cordeiro Sousa, Filipe Brogueira Rodrigues, Gonçalo Duarte, Fátima Campos, Filomena Pinto, A Vaz-Carneiro
OBJECTIVE: Demographic and clinical characteristics associated with nonarteritic anterior ischemic optic neuropathy (NAION) are well described. Patients with hematologic neoplasms may share some of these characteristics, and it may be useful clinically to better understand this set of patients. Our objective is to review systematically the characteristics of patients with both hematologic malignancies and NAION. DESIGN: Systematic review. PARTICIPANTS: Patients with NAION diagnosis related in time to a hematologic neoplasm...
December 2016: Canadian Journal of Ophthalmology. Journal Canadien D'ophtalmologie
https://www.readbyqxmd.com/read/27934876/immunohistochemistry-for-p53-is-a-useful-tool-to-identify-cases-of-acute-myeloid-leukemia-with-myelodysplasia-related-changes-that-are-tp53-mutated-have-complex-karyotype-and-have-poor-prognosis
#9
Sebastian Fernandez-Pol, Lisa Ma, Robert S Ohgami, Daniel A Arber
In this study, we evaluate the expression of p53 in core biopsies with acute myeloid leukemia and correlate the level of expression with acute myeloid leukemia subtype, TP53 mutation status, karyotype, and survival. Of the 143 cases evaluated, 71 fulfilled the WHO 2016 criteria for acute myeloid leukemia with myelodysplasia-related changes, 40 were acute myeloid leukemia-not otherwise specified, 25 were acute myeloid leukemia with recurrent genetic abnormalities, and 7 were therapy-related acute myeloid leukemia...
December 9, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27926581/diagnostic-value-of-platelet-indices-and-bone-marrow-megakaryocytic-parameters-in-immune-thrombocytopenic-purpura
#10
Yue-Ting Tang, Ping He, Ya-Zhen Li, Huan-Zhu Chen, Xiao-Lan Chang, Qing-Dong Xie, Xiao-Yang Jiao
Platelet indices could mirror megakaryopoietic activity in immune thrombocytopenic purpura (ITP), but its specificity and sensitivity need to be studied. The diagnostic performance of platelet indices was analyzed by receiver-operating characteristic curves, and the probability of true positive (sensitivity) and true negative (specificity) in predicting ITP, myelodysplasia, or controls was determined. Mean platelet volume (MPV) was higher, whereas plateletcrit (PCT) was significantly lower in ITP than in myelodysplasia and controls...
December 6, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27872060/bdr-in-newly-diagnosed-patients-with-wm-final-analysis-of-a-phase-2-study-after-a-minimum-follow-up-of-6-years
#11
Maria Gavriatopoulou, Ramón García-Sanz, Efstathios Kastritis, Pierre Morel, Marie-Christine Kyrtsonis, Eurydiki Michalis, Zafiris Kartasis, Xavier Leleu, Giovanni Palladini, Alessandra Tedeschi, Dimitra Gika, Giampaolo Merlini, Pieter Sonneveld, Meletios A Dimopoulos
In this phase 2 multicenter trial, we evaluated the efficacy of the combination of bortezomib, dexamethasone and rituximab (BDR) in 59 previously untreated symptomatic patients with Waldenström's Macroglobulinemia, most of which were of advanced age and with adverse prognostic factors. BDR consisted of a single 21-day cycle of bortezomib alone (1.3 mg/m(2) IV days 1, 4, 8, and 11), followed by weekly IV bortezomib (1.6 mg/m(2) days 1, 8, 15, and 22) for 4 additional 35-day cycles, with IV dexamethasone (40 mg) and IV rituximab (375 mg/m(2)) on cycles 2 and 5, for a total treatment duration of 23 weeks...
November 21, 2016: Blood
https://www.readbyqxmd.com/read/27870069/suspected-myelodysplastic-myeloproliferative-neoplasm-in-a-feline-leukemia-virus-negative-cat
#12
Amy L Weeden, Kyle R Taylor, Scott P Terrell, Alexander E Gallagher, Heather L Wamsley
A 10-year-old castrated Domestic Short-Haired cat was presented to a primary care veterinarian for a wellness examination and laboratory examination for monitoring of diabetes mellitus. The CBC revealed marked thrombocytosis, leukopenia and macrocytic, normochromic anemia. The cat tested negative for FeLV and feline immunodeficiency virus, but was positive for Mycoplasma haemominutum by PCR. Hematologic abnormalities were not responsive to therapy, so a repeat CBC and a bone marrow aspiration for cytology were performed...
December 2016: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/27852504/bilateral-rib-based-distraction-to-the-pelvis-for-the-management-of-congenital-gibbus-deformity-in-the-growing-child-with-myelodysplasia
#13
John T Smith
BACKGROUND: Congenital gibbus deformity of the spine associated with myelodysplasia is a challenging problem in the growing child and is commonly associated with skin breakdown and chronic infection. Surgical solutions including kyphectomy, flap closure of the skin, and early spinal fusion are associated with a high rate of complications and, ultimately, a short trunk due to stoppage of spinal growth. The purpose of this article is to describe our early results in using a rib-based distraction to the pelvis without vertebral resection and fusion to manage this deformity...
January 2016: Spine Deformity
https://www.readbyqxmd.com/read/27820734/the-role-of-the-transcriptional-repressor-growth-factor-independent-1-in-the-formation-of-myeloid-cells
#14
Jennifer Fraszczak, Tarik Möröy
PURPOSE OF REVIEW: Growth factor independent 1 (Gfi1) is a transcriptional repressor that plays multiple roles during myeloid commitment and development. Gfi1-deficient mice lack granulocytes, accumulate aberrant monocytes and show a hyperactivity of macrophages toward bacterial cell wall components. Since these initial findings, numerous additional studies have confirmed the role of Gfi1 in myeloid differentiation from hematopoietic stem cells and multipotent progenitors to bipotential lymphoid/myeloid precursors and myeloid effector cells...
January 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/27800296/klinefelter-syndrome-with-poor-risk-extragonadal-germ-cell-tumor
#15
Jeremy A Konheim, Jonathan A Israel, Scott E Delacroix
Germ cell tumors are the most common malignancy in men aged 15-35 years old, with a small percentage presenting in an extragonadal location. These tumors are seldom identified in the gastrointestinal tract. There is increased risk of extragonadal germ cell tumors (EGCT) in men with Klinefelter syndrome (KS). We report a rare case of a 37-year-old male with KS and EGCT discovered in the duodenum and pelvis. After treatment with Bleomycin-Etoposide-Cisplatin (BEP), he developed growing teratoma syndrome (GTS) and myelodysplasia...
January 2017: Urology Case Reports
https://www.readbyqxmd.com/read/27777803/a-case-of-de-novo-cd5-disseminated-intravascular-large-b-cell-lymphoma-presenting-as-multiorgan-failure
#16
Daulath Singh, Devika Kapuria, Suparna Nanua, Rakesh Gaur
Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months' duration. Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates. The patient later died due to multiorgan failure. A bone marrow biopsy showed 20-30% CD5+ B cells consistent with infiltrative large B-cell lymphoma...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27776050/tibial-rotation-osteotomies-in-a-matched-cohort-of-myelodysplasia-and-cerebral-palsy-children
#17
Peter J Stasikelis, Aaron T Creek, Linda I Wack
BACKGROUND: The purpose of this study is to examine the frequency of complications in children with myelodysplasia (MD) undergoing tibial rotational osteotomies with a matched cohort of children with cerebral palsy (CP). It was postulated that because of the unique health issues facing children with MD more complications would be observed. METHODS: A retrospective chart review was performed to identify children with MD who underwent primary tibial rotational osteotomy between 1997 and 2012 and had a minimum 2-year follow-up...
October 21, 2016: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/27758818/immunophenotypic-analysis-of-erythroid-dysplasia-in-myelodysplastic-syndromes-a-report-from-the-imdsflow-working-group
#18
Theresia M Westers, Eline M P Cremers, Uta Oelschlaegel, Ulrika Johansson, Peter Bettelheim, Sergio Matarraz, Alberto Orfao, Bijan Moshaver, Lisa Eidenschink Brodersen, Michael R Loken, Denise A Wells, Dolores Subira', Matthew Cullen, Jeroen G Te Marvelde, Vincent H J Van der Velden, Frank W M B Preijers, Sung-Chao Chu, Jean Feuillard, Estelle Guérin, Katherina Psarra, Anna Porwit, Leonie Saft, Robin Ireland, Timothy Milne, Marie C Béné, Birgit I Witte, Matteo G Della Porta, Wolfgang Kern, Arjan A van de Loosdrecht
Current recommendations for diagnosing myelodysplastic syndromes endorse flow cytometry as an informative tool. Most flow cytometry protocols focus on the analysis of progenitor cells and the evaluation of the maturing myelomonocytic lineage. However, one of the most frequently observed features of myelodysplastic syndromes is anemia, which may be associated with dyserythropoiesis. Therefore, analysis of changes in flow cytometry features of nucleated erythroid cells may complement current flow cytometry tools...
October 6, 2016: Haematologica
https://www.readbyqxmd.com/read/27749226/update-on-malignancies-in-children-with-juvenile-idiopathic-arthritis-in-the-german-biker-registry
#19
Gerd Horneff, Ariane Klein, Prasad T Oommen, Anton Hospach, Ivan Foeldvari, Isa Feddersen, Kirsten Minden
OBJECTIVES: While tumour necrosis factor (TNF)-α-inhibitor treatment improved outcome of juvenile idiopathic arthritis (JIA) management markedly, concerns have been raised about an association of TNF-α-inhibitor treatment and an increased risk for malignancies especially lymphoma. METHODS: Cases of suspected malignancies documented in the German Biker Registry are reviewed in detail. RESULTS: Until Dec 31, 2015, 3695 JIA patients were prospectively followed with a total of more than 13,198 observation years...
November 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27737699/a-unique-presentation-of-pulmonary-disease-in-advanced-systemic-mastocytosis-proven-by-the-presence-of-mast-cells-in-bronchoalveolar-lavage-a-case-report
#20
Maud A W Hermans, Annemiek Broijl, Paul L A van Daele
BACKGROUND: Systemic mastocytosis is a rare myeloproliferative disease characterized by the uncontrolled proliferation of aberrant mast cells. It has varying clinical manifestations. For unknown reasons, pulmonary localization of mastocytosis is extremely rare. CASE PRESENTATION: In this report, we describe a case of a young Caucasian female with systemic mastocytosis who had an associated hematological non-mast-cell lineage disease with pulmonary interstitial disease directly related to her mastocytosis...
October 13, 2016: Journal of Medical Case Reports
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