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https://www.readbyqxmd.com/read/29353154/cd41-immune-staining-of-micromegakaryocytes-improves-the-diagnosis-of-myelodysplastic-syndrome-and-differentiation-from-pancytopenia
#1
Jinjin Huang, Zhiqiong Wang, Lifang Huang, Miao Zheng
A morphometric analysis was performed on aspirated clots of bone marrow to identify the presence of micromegakaryocytes after immune staining with a monoclonal antibody raised against CD41. Quantitative and qualitative abnormalities of micromegakaryocytes were assessed based on both standard staining and CD41 immune staining. Micromegakaryocytes were largely detected in bone marrow from patients with myelodysplastic syndrome (MDS), while almost no micromegakaryocytes were present in aplastic anemia. CD41 immune staining clearly improved the efficiency of micromegakaryocyte detection under any conditions, showing strong potential as a tool for the auxiliary diagnosis of MDS and differentiation of MDS from pancytopenia, particularly aplastic anemia(AA)...
October 18, 2017: Leukemia Research
https://www.readbyqxmd.com/read/29344583/heterozygous-rtel1-variants-in-bone-marrow-failure-and-myeloid-neoplasms
#2
Judith C W Marsh, Fernanda Gutierrez-Rodrigues, James Cooper, Jie Jiang, Shreyans Gandhi, Sachiko Kajigaya, Xingmin Feng, Maria Del Pilar F Ibanez, Flávia S Donaires, João P Lopes da Silva, Zejuan Li, Soma Das, Maria Ibanez, Alexander E Smith, Nicholas Lea, Steven Best, Robin Ireland, Austin G Kulasekararaj, Donal P McLornan, Anthony Pagliuca, Isabelle Callebaut, Neal S Young, Rodrigo T Calado, Danielle M Townsley, Ghulam J Mufti
Biallelic germline mutations in RTEL1 (regulator of telomere elongation helicase 1) result in pathologic telomere erosion and cause dyskeratosis congenita. However, the role of RTEL1 mutations in other bone marrow failure (BMF) syndromes and myeloid neoplasms, and the contribution of monoallelic RTEL1 mutations to disease development are not well defined. We screened 516 patients for germline mutations in telomere-associated genes by next-generation sequencing in 2 independent cohorts; one constituting unselected patients with idiopathic BMF, unexplained cytopenia, or myeloid neoplasms (n = 457) and a second cohort comprising selected patients on the basis of the suspicion of constitutional/familial BMF (n = 59)...
January 9, 2018: Blood Advances
https://www.readbyqxmd.com/read/29344089/bone-marrow-failure-may-be-caused-by-chromosome-anomalies-exerting-effects-on-runx1t1-gene
#3
R Valli, L Vinti, A Frattini, M Fabbri, G Montalbano, C Olivieri, A Minelli, F Locatelli, F Pasquali, E Maserati
Background: The majority of the cases of bone marrow failure syndromes/aplastic anaemias (BMFS/AA) are non-hereditary and considered idiopathic (80-85%). The peripheral blood picture is variable, with anaemia, neutropenia and/or thrombocytopenia, and the patients with idiopathic BMFS/AA may have a risk of transformation into a myelodysplastic syndrome (MDS) and/or an acute myeloid leukaemia (AML), as ascertained for all inherited BMFS. We already reported four patients with different forms of BMFS/AA with chromosome anomalies as primary etiologic event: the chromosome changes exerted an effect on specific genes, namely RUNX1, MPL, and FLI1, leading to the disease...
2018: Molecular Cytogenetics
https://www.readbyqxmd.com/read/29334367/unmanipulated-haploidentical-transplantation-conditioning-with-busulfan-cyclophosphamide-and-anti-thymoglobulin-for-adult-severe-aplastic-anaemia
#4
L-P Xu, Z-L Xu, F-R Wang, X-D Mo, T-T Han, W Han, Y-H Chen, Y-Y Zhang, J-Z Wang, Y Wang, C-H Yan, Y-Q Sun, F-F Tang, X-H Zhang, X-J Huang
We conducted a retrospective analysis to evaluate outcomes of haploidentical transplantation in adult severe aplastic anaemia (SAA) patients. Fifty-one adults received haploidentical transplantation between May 2011 and December 2016. Patients were administered busulfan (Bu), cyclophosphamide (Cy) and anti-thymoglobulin (ATG) as conditioning regimens, followed by bone marrow and peripheral blood transplantation. The patients' median age was 25 years. Forty-nine patients survived for more than 28 days and all achieved donor myeloid engraftment...
January 15, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29333401/management-of-immune-thrombocytopenia-korean-experts-recommendation-in-2017
#5
REVIEW
Jun Ho Jang, Ji Yoon Kim, Yeung-Chul Mun, Soo-Mee Bang, Yeon Jung Lim, Dong-Yeop Shin, Young Bae Choi, Ho-Young Yhim, Jong Wook Lee, Hoon Kook
Management options for patients with immune thrombocytopenia (ITP) have evolved substantially over the past decades. The American Society of Hematology published a treatment guideline for clinicians referring to the management of ITP in 2011. This evidence-based practice guideline for ITP enables the appropriate treatment of a larger proportion of patients and the maintenance of normal platelet counts. Korean authority operates a unified mandatory national health insurance system. Even though we have a uniform standard guideline enforced by insurance reimbursement, there are several unsolved issues in real practice in ITP treatment...
December 2017: Blood Research
https://www.readbyqxmd.com/read/29330562/influence-of-genetic-polymorphisms-of-il23r-stat3-il12b-and-stat4-on-the-risk-of-aplastic-anemia-and-the-effect-of-immunosuppressive-therapy
#6
Li Zhao, Huanling Zhu, Bing Han, Lixin Wang, Yuming Sun, Xiaojun Lu, Chunyan Huang, Bin Tan, Chunxia Chen, Li Qin
Studies have suggested that IL-23/STAT3 and IL-12/STAT4 signaling pathways associate with aplastic anemia (AA) occurrence. Polymorphisms in pathway-related genes may contribute to AA risk. In the current study, we investigated the association between polymorphisms in genes of IL23R, STAT3, IL12B, and STAT4 and occurrence, severity, and immunosuppressive outcome of AA in the Han population in southwest China. In the current 164 AA cases and 211 controls study, we found T allele and TT genotype of rs7574865 were more frequent in the cases than that in the controls...
January 12, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29321823/yin-and-yang-of-mesenchymal-stem-cells-and-aplastic-anemia
#7
REVIEW
Larisa Broglie, David Margolis, Jeffrey A Medin
Acquired aplastic anemia (AA) is a bone marrow failure syndrome characterized by peripheral cytopenias and bone marrow hypoplasia. It is ultimately fatal without treatment, most commonly from infection or hemorrhage. Current treatments focus on suppressing immune-mediated destruction of bone marrow stem cells or replacing hematopoietic stem cells (HSCs) by transplantation. Our incomplete understanding of the pathogenesis of AA has limited development of targeted treatment options. Mesenchymal stem cells (MSCs) play a vital role in HSC proliferation; they also modulate immune responses and maintain an environment supportive of hematopoiesis...
December 26, 2017: World Journal of Stem Cells
https://www.readbyqxmd.com/read/29316578/acute-hepatitis-as-a-prequel-to-very-severe-aplastic-anemia
#8
Christina Weiler-Normann, Johannes Hartl, Sören Weidemann, Ute-Marie von Pein, Walter Fiedler, Christoph Schramm, Florian Brinkert, Nicolaus Kröger, Maximilian Christopeit
Severe aplastic anemia is a rare and potentially life-threatening disease of the bone marrow often requiring allogeneic hematopoietic stem cell transplantation. Pathogenesis of the disease can vary and often remains enigmatic. Occasionally, severe aplastic anemia is associated with prior severe acute hepatitis. Differential diagnosis of acute non-viral hepatitis challenges the physician as pathogenesis remains unclear.We here present a case of a young patient presenting with acute hepatitis followed by severe aplastic anemia successfully treated with allogeneic hematopoietic stem cell transplantation...
January 2018: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29310957/revascularization-for-aplastic-or-twiglike-middle-cerebral-artery-a-case-report
#9
Yuki Matsunaga, Tsuyoshi Izumo, Yoichi Morofuji, Nobutaka Horie, Kentaro Hayashi, Takayuki Matsuo
An aplastic or twiglike middle cerebral artery (Ap/T-MCA) is a congenital anomaly related to interference of normal embryonic development of the middle cerebral artery (MCA). This condition may cause ischemic and hemorrhagic strokes as a result of hypoperfusion, vulnerable collateral circulation, and associated aneurysms. We reported a case of Ap/T-MCA with transient ischemic attack, which was successfully treated with revascularization. A 19-year-old woman with a history of transient left upper limb hemiparesis, dysesthesia, and headache visited our hospital...
January 5, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29309293/orthopaedic-manifestations-of-sickle-cell-disease
#10
Kelly L Vanderhave, Crystal A Perkins, Brian Scannell, Brian K Brighton
Sickle cell disease (SCD) is an autosomal recessive disorder that results in hemolytic anemia related to abnormal hemoglobin and erythrocyte levels. SCD is characterized by vascular occlusive episodes, visceral sequestration, and aplastic or hemolytic crises. These crises most commonly occur in bone. The orthopaedic manifestations of SCD comprise much of the morbidity associated with this disorder. Osteonecrosis and osteomyelitis are among the most disabling and serious musculoskeletal complications in patients with SCD...
January 5, 2018: Journal of the American Academy of Orthopaedic Surgeons
https://www.readbyqxmd.com/read/29307285/incidence-of-fanconi-anaemia-in-phenotypically-normal-aplastic-anaemia-patients-in-west-bengal
#11
Atreyee Dutta, Rajib De, Tuphan Kanti Dolai, Pritha Pal, Shanoli Ghosh, Pradip Kumar Mitra, Ajanta Halder
OBJECTIVES: Fanconi anaemia (FA) is a rare inherited bone marrow failure and autosomal recessive blood disorder. FA patients have a higher risk of cancer, including acute myeloid leukaemia and squamous cell carcinoma. Maximum, but not all, affected individuals have one or more somatic abnormalities, including skin, skeletal, genitourinary, gastrointestinal, cardiac and neurological anomalies, etc. Positive stress cytogenetics has immense implications for the treatment and management of FA...
January 7, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29306655/comparative-study-of-porcine-anti-human-lymphocyte-immunoglobulin-and-rabbit-anti-human-thymocyte-immunoglobulin-as-a-first-line-treatment-of-acquired-severe-aplastic-anemia
#12
Miao Chen, Chao Liu, Xinhui Qiao, Daobin Zhou, Junling Zhuang, Bing Han
Porcine anti-human lymphocyte immunoglobulin (pALG) and rabbit anti-human thymocyte immunoglobulin (rATG) are the only two ATGs for severe aplastic anemia (SAA) treatment in China. 148 treatment-naïve SAA patients who received ATG combined with cyclosporine A (CsA) therapy were analysed retrospectively. The patients were divided into a pALG group (n = 114) and a rATG group (n = 34). After three months, the pALG and rATG groups had an overall response (OR) of 65.8% and 44.1%, respectively (P = 0...
January 2, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29303078/molecular-adjuvants-based-on-plasmids-encoding-protein-aggregation-domains-affect-bone-marrow-niche-homeostasis
#13
Maria Giovanna Sabbieti, Giovanna Lacava, Andrea Amaroli, Luigi Marchetti, Roberta Censi, Piera Di Martino, Dimitrios Agas
BACKGROUND: During last years, DNA vaccine immunogenicity has been optimized by the employment of co-stimulatory molecules and molecular adjuvants. It has been reported that plasmid (pATRex), encompassing the DNA sequence for the von Willebrand A (vWA/A) domain of the Anthrax Toxin Receptor-1 (ANTXR-1, alias TEM8, Tumor Endothelial Marker 8), acts as strong immune adjuvant by inducing formation of insoluble intracellular aggregates. Markedly, we faced with upsetting findings regarding the safety of pATRex as adjuvant since the aggregosome formation prompted to osteopenia in mice...
January 5, 2018: Current Gene Therapy
https://www.readbyqxmd.com/read/29303047/eltrombopag-in-the-management-of-aplastic-anaemia-real-world-experience-in-a-non-trial-setting
#14
Yu-Yan Hwang, Harinder Gill, Thomas S Y Chan, Garret M K Leung, Carol Y M Cheung, Yok-Lam Kwong
OBJECTIVE: The thrombopoietin mimetic eltrombopag has been used in clinical trials for the frontline and salvage treatment of aplastic anaemia (AA). Eltrombopag was investigated in AA patients on a non-trial all-comer basis. METHODS: Consecutive newly diagnosed and relapsed/refractory AA patients were treated with eltrombopag. RESULTS: In a 4.5-year period, 20 consecutive AA patients (newly diagnosed, N = 10; relapsed/refractory, N = 10) at a median age of 47 (22-84) years were treated with eltrombopag...
January 5, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29301129/amegakaryocytic-thrombocytopenia-and-subsequent-aplastic-anemia-associated-with-apparent-epstein-barr-virus-infection
#15
Ilana Levy, Ruth Laor, Nizar Jiries, Jacob Bejar, Aaron Polliack, Tamar Tadmor
Acquired amegakaryocytic thrombocytopenia (AAT), a rare entity characterized by severe thrombocytopenia and the absence of megakaryocytes in the bone marrow, may mimic or precede the diagnosis of aplastic anemia (AA). Here, we describe a patient who presented with apparent Epstein-Barr virus (EBV)-associated immune thrombocytopenia resistant to several lines of therapies, which was in fact a form of AAT with some features of AA. He eventually responded to therapy with eltrombopag, cyclosporine A (CSA), and antithymocyte globulin (ATG) and recovered completely...
January 5, 2018: Acta Haematologica
https://www.readbyqxmd.com/read/29297952/first-line-choice-for-severe-aplastic-anemia-in-children-transplantation-from-a-haploidentical-donor-vs-immunosuppressive-therapy
#16
Yifei Cheng, Zhengli Xu, Yuanyuan Zhang, Jun Wu, Fengrong Wang, Xiaodong Mo, Yuhong Chen, Wei Han, Jinsong Jia, Yu Wang, Xiaohui Zhang, Xiaojun Huang, Leping Zhang, Lanping Xu
We retrospectively compared the outcomes of children with severe aplastic anemia (SAA) who received immunosuppressive therapy (IST) or who underwent hematopoietic stem cell transplantation (HSCT) from a haploidentical donor (HID), between 2007 and 2016. A total of 52 children with SAA under the age of 17 years were initially treated with IST (n = 24) or haploidentical HSCT (n = 28) as first-line treatment. The estimated 10-year overall survival was 73.4 ± 12.6% and 89.3 ± 5.8% in patients treated with IST or HID-HSCT (P = ...
December 18, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/29296774/treated-secondary-acute-myeloid-leukemia-a-distinct-high-risk-subset-of-aml-with-adverse-prognosis
#17
Prajwal Boddu, Hagop M Kantarjian, Guillermo Garcia-Manero, Farhad Ravandi, Srdan Verstovsek, Elias Jabbour, Gautam Borthakur, Marina Konopleva, Kapil N Bhalla, Naval Daver, Courtney D DiNardo, Christopher B Benton, Koichi Takahashi, Zeev Estrov, Sherry R Pierce, Michael Andreeff, Jorge E Cortes, Tapan M Kadia
Secondary acute myeloid leukemia (s-AML) includes therapy-related AML and AML evolving from antecedent hematological disorder (AHD). s-AML arising after treating AHD likely represents a prognostically distinct, high-risk disease category. In this study, treated s-AML (ts-AML) was defined by: (1) prior diagnosis of myelodysplasia, myeloproliferative neoplasm, or aplastic anemia and (2) at least 1 therapy for that diagnosis. ts-AML was categorized by age (< or ≥60 years), and each cohort assessed for response rates and overall survival (OS) on various treatment regimens...
July 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/29290359/nutritional-support-contributes-to-recuperation-in-a-rat-model-of-aplastic-anemia-by-enhancing-mitochondrial-function
#18
Guang Yang, Lifen Zhao, Bing Liu, Yujia Shan, Yang Li, Huimin Zhou, Li Jia
OBJECTIVES: Acquired aplastic anemia (AA) is a hematopoietic stem cell disease that leads to hematopoietic disorder and peripheral blood pancytopenia. We investigated whether nutritional support is helpful to AA recovery. METHODS: We established a rat model with AA. A nutrient mixture was administered to rats with AA through different dose gavage once per day for 55 d. Animals in this study were assigned to one of five groups: normal control (NC; group includes normal rats); AA (rats with AA); high dose (AA + nutritional mixture, 2266...
February 2018: Nutrition
https://www.readbyqxmd.com/read/29281994/successful-treatment-of-cerebral-aspergillosis-case-report-of-a-patient-with-t-cell-large-granular-lymphocytic-leukemia-t-lgl
#19
Amin T Turki, Jassin Rashidi-Alavijeh, Jan Dürig, Guido Gerken, Peter-Michael Rath, Oliver Witzke
BACKGROUND: Invasive aspergillosis involving patients with neutropenia or severe immunosuppression, such as patients with hematologic malignancies is associated with high mortality. Patients with T-cell large granular lymphocytic leukemia (T-LGL) on the other hand are considered to be less vulnerable for severe opportunistic fungal infection as their course of disease is chronic and marked by less violent cytopenia then in e.g. Aplastic Anemia. Only neutropenia is regarded as independent risk factor for severe opportunistic infection in T-LGL patients...
December 28, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/29262476/-short-stature-optic-nerve-atrophy-and-pelger-hu%C3%A3-t-anomaly-syndrome-with-antibody-immunodeficiency-and-aplastic-anemia-a-case-report-and-literature-review
#20
T Y He, N Zhang, Y Xia, Y Luo, C R Li, J Yang
Objective: To investigate the clinical features and genetic characteristics of cases with NBAS gene defects. Method: Characteristics of clinical materials, immunological data and gene mutation of the first case in China with NBAS gene mutation were retrospectively analyzed. The related literature was searched by using search terms'NBAS'. Result: A 2-year-four-month old girl, was admitted due to 'fever and pallor for one day'. There was an intrauterine growth retardation at her fetal stage. Since her birth, she had suffered from recurrent infections and development delay was accompanied by persistent liver dysfunction...
December 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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