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Aplastic anemia

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https://www.readbyqxmd.com/read/29665934/-effect-of-regulating-ppar%C3%AE-by-mtor-signaling-on-adipogenesis-of-bone-marrow-mesenchymal-stem-cells-from-aplastic-anemia
#1
Fang Wei, Wei-Huan Zhang, Xiu-Lian Zhang, Xiao-Jing Ge, Shu-Qin Zhang
OBJECTIVE: To study the effect and mechanism of mTOR signaling on adipogenesis of bone marrow mesenchymal stem cells(BM-MSCs) from aplastic anemia (AA) patients through regulation of PPARγ. METHODS: BM-MSCs were isolated from 24 newly diagnosed AA patients and 24 healthy controls. The surface antigen expression of BM-MSCs was identified by flow cytometry. The capacity of adipogenic differentiation of BM-MSCs was determined by lipid droplets based on Oil Red O staining and by the expression of FABP4 based on Western blot...
April 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29665923/-the-effect-of-serum-cytokine-levels-prior-transplantation-on-the-outcome-of-severe-aplastic-anemia-patients-received-allogeneic-hematopoietic-stem-cell-transplantation
#2
Zhao Wang, Yuan-Yuan Shi, Xin Yang, Su-Dong Zhang, Li-Ning Zhang, Dong-Lin Yang, Yi He, Rong-Li Zhang, Er-Lie Jiang, Jia-Lin Wei, Si-Zhou Feng, Min-Zhe Han
OBJECTIVE: To identify the role of serum cytokine levels prior allogeneic hematopoietic stem cell trans-plantation (allo-HSCT) in the outcome of severe aplastic anemia (SAA) patients received allo-HSCT treatment. METHODS: The clinical data of 117 SAA patients received allo-HSCT were enrolled in this study. The overall survival (OS), graft versus host disease (GVHD) incidence and relationship of serum cytokines with OS and major transplantation complications were retrospectively analyzed...
April 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29664113/peripartum-management-of-hla-alloimmune-platelet-refractoriness
#3
Jeremy Ryan Peña, Leanna Sudhof, Barbara O'Brien
BACKGROUND: Platelet (PLT) refractoriness presents a challenging problem for transfusion support, especially in the perioperative setting, where there is urgency for human leukocyte antigen (HLA)-compatible units, yet identification and provision of compatible PLT concentrates requires time. CASE REPORT: A 22-year-old G3P1 woman with thrombocytopenia due to aplastic anemia, likely autoimmune, presented in her third trimester for peripartum care and newly diagnosed HLA alloimmune PLT refractoriness...
April 17, 2018: Transfusion
https://www.readbyqxmd.com/read/29663858/assessment-of-liver-and-cardiac-iron-overload-using-mri-in-patients-with-chronic-anemias-in-latin-american-countries-results-from-asimila-study
#4
Rodolfo Cancado, Nora P Watman, Clarisse Lobo, Zulay Chona, Fernando Manzur, Fabiola Traina, Miriam Park, Guillermo Drelichman, Juan Pablo Zarate, Luis Marfil
OBJECTIVES: A multicenter, noninterventional, observational study was conducted in the Latin American countries including Argentina, Brazil, Colombia, Mexico, and Venezuela to assess the prevalence of liver and cardiac iron overload using magnetic resonance imaging (MRI) in patients with chronic anemias except thalassemia. METHODS: Patients aged >10 years with transfusion-dependent anemias, except thalassemia, either with <20 units of red blood cell (RBC) transfusions with serum ferritin (SF) levels >2000 ng/mL or with ≥20 units of RBC transfusions regardless of SF level in their lifetime, were enrolled...
April 17, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29663189/a-characteristic-flow-cytometric-pattern-with-broad-forward-scatter-and-narrowed-side-scatter-helps-diagnose-immune-thrombocytopenia-itp
#5
Raita Araki, Ryosei Nishimura, Rie Kuroda, Toshihiro Fujiki, Shintaro Mase, Kazuhiro Noguchi, Yasuhiro Ikawa, Hideaki Maeba, Akihiro Yachie
Various disorders cause severe thrombocytopenia, which can lead to critical hemorrhage. Procedures that rapidly support the diagnosis and risk factors for serious bleeding were explored, with a focus on immune thrombocytopenia (ITP). Twenty-five patients with thrombocytopenia, including 13 with newly diagnosed ITP, 3 with chronic ITP, 6 with aplastic anemia (AA), and 3 with other thrombocytopenia (one acute myeloid leukemia, one acute lymphoblastic leukemia, and one hemophagocytic lymphohistiocytosis), were reviewed...
April 16, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29653202/clinicopathological-feature-and-outcome-of-pythiosis
#6
Maria Nina Chitasombat, Noppadol Larbcharoensub, Ariya Chindamporn, Theerapong Krajaejun
OBJECTIVES: Vascular pythiosis is a life-threatening infection, caused by Pythium insidiosum. We presented the clinical presentation, serodiagnosis, pathology, and outcome from our institution. METHODS: We retrospectively analyzed patients with proven vascular pythiosis at Ramathibodi Hospital, Mahidol University, Bangkok, Thailand from January 2006 to December 2016. RESULTS: Thirteen patients were analyzed, eight thalassemias. Five had aplastic anemia, myelodysplasia, acute leukemia, cirrhosis, and alcoholism...
April 10, 2018: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/29650274/oral-cancer-in-fanconi-anemia-review-of-121-cases
#7
REVIEW
Camila Pinheiro Furquim, Allana Pivovar, José Miguel Amenábar, Carmem Bonfim, Cassius Carvalho Torres-Pereira
Fanconi anemia (FA) is a rare autosomal recessive genetic disorder characterized by aplastic anemia, progressive pancytopenia, congenital anomalies, and increased risk of cancer development. After hematopoietic stem cell transplant (HSCT), patients have an estimated 500-fold increase in the risk of developing head and neck cancer compared to a non-affected, and the oral cavity is affected in one-third of cases. Thus, this study aimed to better understand the natural history of oral cavity cancer in patients affected by FA...
May 2018: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/29620676/hepatitis-associated-aplastic-anemia-treated-successfully-with-antithymocyte-globulin
#8
Hongyan Ji, Li Chen, Xiaojun Sun, Xiuli Li, Wei Chen, Ping Zhao, Yulin Wang
Hepatitis-associated aplastic anemia (HAAA) is a variant of acquired aplastic anemia in which bone marrow failure follows the development of an acute episode of seronegative hepatitis. HAAA occurs most frequently in male children and is lethal if left untreated. Antilymphocyte globulin, antithymocyte globulin, and allogeneic bone marrow transplantation have been used in the treatment of this disease. In this work, we report the case of a 3-year-old boy with HAAA treated successfully with immunosuppressive therapy...
April 3, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29611722/single-nucleotide-polymorphisms-of-fas-and-fasl-genes-and-risk-of-idiopathic-aplastic-anemia
#9
Sadia Rehman, Nusrat Saba, Madiha Naz, Parvez Ahmed, Saeeda Munir, Sumaira Sajjad, Sobia Tabassum, Lubna Naseem
FAS/FASL signaling system plays a vital role in the regulation of apoptosis, envisaged as a death process required for immune surveillance to prevent autoimmunity and tumorigenesis along with several other biological activities. Several single-nucleotide polymorphisms (SNPs) of FAS/FASL system can result in aberrant apoptosis, which can cause different cancers and autoimmune diseases. Aplastic anemia (AA) is an autoimmune dysfunction characterized by peripheral blood pancytopenia associated with hypoplasia of bone marrow...
April 3, 2018: Immunological Investigations
https://www.readbyqxmd.com/read/29596882/swi-snf-subunit-expression-heterogeneity-in-human-aplastic-anemia-stem-progenitors
#10
Sayantani Sinha, Shankha Subhra Chatterjee, Mayukh Biswas, Arijit Nag, Debasis Banerjee, Rajib De, Amitava Sengupta
Acquired aplastic anemia (AA) is a bone marrow failure that is associated with auto-immune destruction of hematopoietic stem cells (HSCs). Although somatic mutations have been identified in AA patients, mutations alone would not suffice understanding AA pathophysiology. SWI/SNF is an evolutionarily conserved, multi-subunit, ATP-dependent chromatin remodeling protein complex that plays important role in mammalian hematopoiesis. Herein, gene expression analysis identified a significant loss of SWI/SNF core component SMARCC1 along with ARID1B, ACTL6A and SMARCD1 in human AA bone marrow CD34+ HSC and progenitors (HSPCs) compared to normal HSPCs...
March 26, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29590742/myasthenia-gravis-associated-with-thymoma-and-aplastic-anemia-case-report
#11
Lidija Dežmalj Grbelja, Radovan Vrhovac, Monika Ulamec
Myasthenia gravis is associated in 10 to 15 percent of patients with thymic tumors, rarely with aplastic anemia. We report a 45-year-old male diagnosed with myasthenia gravis -associated with thymoma. We started treatment with pyridostigmine. After thymectomy, the patient -received 30 irradiation sessions. In the postoperative course, he had mild worsening of myasthenia gravis, which improved with prednisone. Five months later, he developed severe aplastic anemia. He was dependent on blood supplement. After allogeneic transplantation of bone marrow, he improved but later he -developed graft versus host disease...
December 2017: Acta Clinica Croatica
https://www.readbyqxmd.com/read/29588856/diagnosis-of-gata2-haplo-insufficiency-in-a-young-woman-prompted-by-pancytopenia-with-deficiencies-of-b-cell-and-dendritic-cell-development
#12
Allen Sanyi, David L Jaye, Cecilia B Rosand, Amanda Box, Chandrakasan Shanmuganathan, Edmund K Waller
Background: GATA2 deficiency presents with a spectrum of phenotypes including increased susceptibility to viral and bacterial infections, multi-lineage cytopenias, aplastic anemia, leukemic transformation and lymphedema. Allogeneic transplantation is only curative therapy for GATA2 deficiency, but is associated with significant treatment related morbidity and mortality. Given the spectrum of clinical presentation, accurate diagnosis of GATA2 deficiency is necessary to identify patients early in their disease course when allogeneic bone marrow transplantation may be of clinical benefit...
2018: Biomarker Research
https://www.readbyqxmd.com/read/29584887/national-survey-of-myeloablative-total-body-irradiation-prior-to-hematopoietic-stem-cell-transplantation-in-japan-survey-of-the-japanese-radiation-oncology-study-group-jrosg
#13
Naoya Ishibashi, Toshinori Soejima, Hiroki Kawaguchi, Takeshi Akiba, Masatoshi Hasegawa, Kouichi Isobe, Hitoshi Ito, Michiko Imai, Yasuo Ejima, Masaharu Hata, Keisuke Sasai, Emiko Shimoda, Toshiya Maebayashi, Masahiko Oguchi, Tetsuo Akimoto
A myeloablative regimen that includes total-body irradiation (TBI) before hematopoietic stem cell transplantation results in higher patient survival rates than achieved with regimens without TBI. The TBI protocol, however, varies between institutions. In October 2015, the Japanese Radiation Oncology Study Group initiated a national survey of myeloablative TBI (covering 2010-2014). Among the 186 Japanese institutions performing TBI, 90 (48%) responded. The 82 institutions that had performed myeloablative TBI during this period treated 2698 patients with malignant disease [leukemia (2082 patients, 77...
March 23, 2018: Journal of Radiation Research
https://www.readbyqxmd.com/read/29573075/plasmodium-falciparum-infection-transmitted-by-transfusion-a-cause-of-hemophagocytic-syndrome-after-bone-marrow-tranplantation-in-a-non-endemic-country
#14
Saloua Ladeb, Nour Ben Abdejlil, Najla Fakhfakh, Amel Lakhal, Dorra Belloumi, Leila Ben Hamed, Aicha Kallel, Lamia Torjman, Rym El Fatimi, Slama Hmida, Kalthoum Kallel, Tarek Ben Othman
A 27-year-old man with severe aplastic anemia underwent bone marrow transplantation from his HLA identical brother in July 2016. Conditioning included ATGAM 30 mg/kg for 3 days and Cyclophosphamide 50 mg/kg for 4 days. The patient received several platelet and red blood cell transfusions before and after the conditioning. The patient received broad spectrum antibiotics and caspofungin because persistant febrile neutropenia without bacteriological or mycological documentation. Hemophagocytic syndrome was diagnosed on day +12...
March 23, 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29571658/cryopreservation-of-testicular-tissue-in-pre-pubertal-and-adolescent-boys-at-risk-for-infertility-a-low-risk-procedure
#15
Jessica M Ming, Michael E Chua, Roberto Iglesias Lopes, Anne Marie Maloney, Abha A Gupta, Armando J Lorenzo
INTRODUCTION: Cryopreservation of testicular tissue (TT) has become an increasingly attractive option for fertility preservation (FP), particularly for pre-pubertal boys at risk for gonadotoxicity from cancer therapy. At our institution, all at-risk families undergo counseling regarding infertility risk and available FP strategies, including this vulnerable patient population. As the technology required to use the acquired tissue is, as yet, unproven, it is paramount to document minimal morbidity and complications from this procedure...
March 9, 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29568696/aplastic-anemia-secondary-to-nivolumab-and-ipilimumab-in-a-patient-with-metastatic-melanoma-a-case-report
#16
D E Meyers, W F Hill, A Suo, V Jimenez-Zepeda, T Cheng, N A Nixon
Background: Immune checkpoint blockade (ICB) is becoming an increasingly prevalent strategy in the clinical realm of cancer therapeutics. With more patients being administered ICB for a host of tumor types, the scope of adverse events associated with these drugs will likely grow. Here we report a case of aplastic anemia (AA) in a patient with metastatic melanoma secondary to dual ICB therapy. To our knowledge, this is only the second case of AA secondary to dual ICB in the literature, and the first to have a positive patient outcome...
2018: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/29564940/quantitative-measurement-of-oxidative-damage-in-erythrocytes-as-indicator-in-benzene-intoxications
#17
José Luis Martínez-Rodríguez, Rosalinda Gutiérrez-Hernández, Claudia Araceli Reyes-Estrada, Angelica Judith Granados-López, Tatiana Arcos-Ortega, Jesús Adrián López
The metabolism of aromatic hydrocarbons by the organism forms products that cause cell death depending on the type of exposure. Benzene exposure has been linked to oxidative stress, hepatic damage, aplastic anemia and hematopoietic cancer as lymphoid and myeloid leukemia. However, there are not fast methods to evaluate chronically benzene exposure in human blood. The objective of this work was the evaluation of the correlation between oxidative damage with benzene exposure and the level of Cellular Plasma Membrane Stability (CPMS) in erythrocytes to use it as a future indicator to determine the grade of benzene intoxications...
March 22, 2018: Toxicology Mechanisms and Methods
https://www.readbyqxmd.com/read/29564363/acute-epiglottitis-in-the-immunocompromised-host-case-report-and-review-of-the-literature
#18
REVIEW
Cheng Chen, Mukil Natarajan, David Bianchi, Georg Aue, John H Powers
We present a case of acute epiglottitis in a 16-year-old with severe aplastic anemia. He was admitted with a history suggestive of a severe upper airway infection and an absolute neutrophil count of 0 per cubic millimeter. Despite his immunocompromised state, he presented with the classical signs and symptoms of epiglottitis. We review here the presentation and comorbidities of immunocompromised patients with epiglottitis. In addition, the appropriate choice of empirical antibiotic therapy is important for the management of epiglottitis in immunocompromised patients, especially in the post- Haemophilus influenza type B vaccination era...
March 2018: Open Forum Infectious Diseases
https://www.readbyqxmd.com/read/29563590/successful-outcomes-of-second-hematopoietic-stem-cell-transplantation-with-total-nodal-irradiation-and-atg-conditioning-for-graft-failure-in-adult-patients-with-severe-aplastic-anemia
#19
Seung-Ah Yahng, Sung-Soo Park, Young-Woo Jeon, Jae-Ho Yoon, Seung-Hwan Shin, Sung-Eun Lee, Byung-Sik Cho, Ki-Seong Eom, Yoo-Jin Kim, Seok Lee, Chang-Ki Min, Hee-Je Kim, Seok-Goo Cho, Dong-Wook Kim, Woo-Sung Min, Jong Wook Lee
Data regarding the optimal approach for second allogeneic hematopoietic stem cell transplantation (HSCT) after graft failure (GF) in acquired severe aplastic anemia (SAA) are still limited and heterogeneous. We examined 24 patients who underwent second HLA-matched sibling donor (MSD) peripheral blood HSCT for GF. The reconditioning regimen (TNI-750/ATG) consisted of a single dose of total nodal irradiation (TNI, 750 cGy) and antithymocyte globulin (ATG; Thymoglobulin® , 1.25 mg/kg/day for 3 days). All but one patient achieved successful engraftment of neutrophils (median 12 days, range 5-21) and platelets (median 15 days, range 9-316)...
March 21, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29562777/placenta-derived-adherent-stromal-cell-therapy-for-hematopoietic-disorders-a-case-study-of-plx-r18
#20
Noa Sher, Racheli Ofir
The ephemeral placenta provides a noncontroversial source of young, healthy cells of both maternal and fetal origin from which cell therapy products can be manufactured. The 2 advantages of using live cells as therapeutic entities are: (a) in their environmental-responsive, multifactorial secretion profile and (b) in their activity as a "slow-release drug delivery system," releasing secretions over a long time frame. A major difficulty in translating cell therapy to the clinic involves challenges of large-scale, robust manufacturing while maintaining product characteristics, identity, and efficacy...
January 2018: Cell Transplantation
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