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https://www.readbyqxmd.com/read/29778393/fertility-preservation-in-pediatric-female-cancer-patients
#1
Sara E Arian, Rebecca L Flyckt, Richard Herman, Hadi Erfani, Tommaso Falcone
OBJECTIVE: To describe and demonstrate ovarian tissue cryopreservation (OTC) as an emerging fertility preservation technique DESIGN: Video presentation. SETTING: University hospital. PATIENT(S): A 6 year-old female patient diagnosed with aplastic anemia with plan for bone marrow transplantation underwent laparoscopic unilateral oophorectomy in conjunction with surgical procedure for port placement by the pediatric surgeon, followed by cryopreservation of ovarian tissue...
May 2018: Fertility and Sterility
https://www.readbyqxmd.com/read/29773597/hematopoietic-stem-cell-loss-and-hematopoietic-failure-in-severe-aplastic-anemia-is-driven-by-macrophages-and-aberrant-podoplanin-expression
#2
Amanda McCabe, Julianne N P Smith, Angelica Costello, Jackson Maloney, Divya Katikaneni, Katherine C MacNamara
Severe aplastic anemia results from profound hematopoietic stem cell loss. T cells and interferon gamma have long been associated with severe aplastic anemia, yet the underlying mechanisms driving hematopoietic stem cell loss remain unknown. Using a mouse model of severe aplastic anemia, we demonstrate that interferon gamma-dependent hematopoietic stem cell loss required macrophages. Interferon gamma was necessary for bone marrow macrophage persistence, despite loss of other myeloid cells and hematopoietic stem cells...
May 17, 2018: Haematologica
https://www.readbyqxmd.com/read/29772350/unmanipulated-haploidentical-hematopoietic-stem-cell-transplantation-achieved-outcomes-comparable-with-matched-unrelated-donor-transplantation-in-young-acquired-severe-aplastic-anemia
#3
Yue Lu, Rui-Juan Sun, Yan-Li Zhao, Min Xiong, Xing-Yu Cao, Jing-Pin Zhang, Zhi-Jie Wei, Jia-Rui Zhou, De-Yan Liu, Dao-Pei Lu
Salvage haploidentical HSCT is considered in patients with severe aplastic anemia (SAA) if matched unrelated donor (MUD) is unavailable. However, studies on haplo- and MUD transplantation in SAA are yet lacking. The present study retrospectively analyzed the outcomes of 89 young SAA patients who underwent unmanipulated alternative hematopoietic stem cell transplantation (HSCT) between September 2012 and September 2016 at our single center. 41 patients received haploidentical donors, and 48 patients received MUD for HSCT...
May 14, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29760547/comparison-of-the-effects-of-deferasirox-deferoxamine-and-combination-of-deferasirox-and-deferoxamine-on-an-aplastic-anemia-mouse-model-complicated-with-iron-overload
#4
Dijiong Wu, Xiaowen Wen, Wenbin Liu, Huijin Hu, Baodong Ye, Yuhong Zhou
Background and aim: Iron overload is commonly observed during the course of aplastic anemia (AA), which is believed to aggravate hematopoiesis, cause multiple organ dysfunction, lead to disease progression, and impair quality of life. Deferasirox (DFX) and deferoxamine (DFO) are among the most common iron chelation agents available in the clinical setting. The aim of this study was to investigate if the combination therapy with DFX and DFO is superior in hematopoietic recovery and iron chelation...
2018: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/29760162/unrelated-cord-blood-transplantation-in-patients-with-acquired-refractory-aplastic-anemia-a-nationwide-phase-ii-study
#5
Regis Peffault de Latour, Sylvie Chevret, Charlotte Jubert, Anne Sirvent, Claire Galambrun, Annalisa Ruggeri, Virginie Gandemer, Jérôme Cornillon, Fanny Rialland, Jean-Hugues H Dalle, Edouard Forcade, Benedicte Bruno, Catherine Paillard, Pierre S Rorlich, Alexandra Salmon, Sabine Fürst, Flore Sicre de Fontbrune, Marie Therese Rubio, Jacques-Olivier Bay, Mohamad Mohty, Jerome Larghero, Eliane Gluckman, Gerard Socié
Outcomes remain poor for patients with refractory severe aplastic anemia (SAA). Alternative donor transplantation may be considered, but results from previous studies have not been encouraging. We conducted a prospective nationwide phase II study to assess the efficacy and safety of unrelated cord blood transplantation (CBT) in patients with refractory SAA (APCORD protocol, NCT 01343953). To demonstrate a significant difference in one-year survival from 20% (null hypothesis) to 50% (alternative hypothesis), we needed to include 25 transplanted patients...
May 14, 2018: Blood
https://www.readbyqxmd.com/read/29749397/recurrent-somatic-mutations-are-rare-in-patients-with-cryptic-dyskeratosis-congenita
#6
Martin Kirschner, Angela Maurer, Marcin W Wlodarski, Monica S Ventura Ferreira, Anne-Sophie Bouillon, Insa Halfmeyer, Wolfgang Blau, Michael Kreuter, Martin Rosewich, Selim Corbacioglu, Joachim Beck, Michaela Schwarz, Jörg Bittenbring, Markus P Radsak, Christian Matthias Wilk, Steffen Koschmieder, Matthias Begemann, Ingo Kurth, Mirle Schemionek, Tim H Brümmendorf, Fabian Beier
Dyskeratosis congenita (DKC) is a paradigmatic telomere disorder characterized by substantial and premature telomere shortening, bone marrow failure, and a dramatically increased risk of developing myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). DKC can occur as a late-onset, so-called cryptic form, with first manifestation in adults. Somatic MDS-related mutations are found in up to 35% of patients with acquired aplastic anemia (AA), especially in patients with short telomeres. The aim of our study was to investigate whether cryptic DKC is associated with an increased incidence of MDS-related somatic mutations, thereby linking the accelerated telomere shortening with the increased risk of MDS/AML...
April 2, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29749240/why-is-it-necessary-to-examine-retina-when-the-patient-suffers-from-aplastic-anemia
#7
D Tomcikova, A Gerinec, B Busanyova, M Gresikova, S Biskup, K Hortnagel
PURPOSES: To inform about a case of Revesz syndrome (RS) with initial ophthalmological symptomatology of severe proliferative vitreoretinopathy of the left eye (LE). After the aplastic anemia had developed, RS was established. The exudative retinopathy was successfully treated with photocoagulation on the right eye (RE). BACKGROUND: RS is characterized by fatal bone marrow failure, exudative retinopathy, neuroradiographic abnormalities, neurodevelopmental delay and skin abnormalities...
2018: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/29744996/hematopoietic-stem-cell-transplantation-without-in-vivo-t-cell-depletion-for-pediatric-aplastic-anemia-a-single-center-experience
#8
Sidan Li, Bin Wang, Lingling Fu, Yilin Pang, Guanghua Zhu, Xuan Zhou, Jie Ma, Yan Su, Maoquan Qin, Runhui Wu
For young patients, HLA-MRD HSCT is the first-line treatment of SAA. However, due to China's birth control policy, few patients could find suitable sibling donors and HLA-MUD. More and more transplantation centers have used Haplo-D as the donor source for young adult and pediatric patients. However, studies with larger amount of pediatric patients are rare. We retrospectively analyzed the data of children with AA who were treated with allogeneic HSCT and compared the therapeutic efficacy of Haplo-HSCT and MRD/MUD group...
May 10, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29743401/-hypoplastic-acute-promyelocytic-leukemia-with-continuous-hypocellular-bone-marrow-after-remission
#9
Toshiki Nakamura, Junya Makiyama, Ayumi Matsuura, Hirokazu Kurohama, Hideaki Kitanosono, Masahiro Ito, Shinichiro Yoshida, Yasushi Miyazaki
An 87-year old female presented with unsteady gait and occasional subcutaneous hematomas. Blood examination findings revealed pancytopenia and mild coagulopathy. Both the histopathological evaluation of bone marrow smears and bone marrow biopsy revealed a hypocellular bone marrow. However, APL cells were observed and PML-RARA fusion gene was detected. On the basis of these findings, the patient was diagnosed with hypoplastic acute promyelocytic leukemia. She received ATRA treatment and achieved complete remission (CR) 29 days from the commencement of therapy...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29743400/-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-subsequent-to-rabbit-antithymocyte-globulin-administration-and-successfully-treated-with-rituximab-in-a-patient-with-aplastic-anemia
#10
Hiroaki Nagata, Takahiro Fujino, Ayako Muramatsu, Yuka Kawaji, Kodai Kuriyama, Muneo Oshiro, Yoshiko Hirakawa, Toshiki Iwai, Hitoji Uchiyama
Rabbit antithymocyte globulin (ATG) is an effective immunosuppressive therapy for patients with aplastic anemia (AA). However, Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) is a rare but serious complication of the therapy. An 81-year-old man was diagnosed with severe AA on the occasion of melena. Because cyclosporine monotherapy did not improve his condition, rabbit ATG was additionally administered. Thirty-one days after the administration of rabbit ATG, the patient presented with fever and general malaise...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29739053/fungal-infection-caused-by-geotrichum-capitatum-in-a-severe-aplastic-anemia-patient-a-case-report-and-review-of-the-literature
#11
Wulin Shan, Chunyang Dai, Jingsong Kan, Ming Li, Meiling Yin
BACKGROUND: Invasive fungal infections often occur in immunocompromised patients. Here, we report an infection case caused by Geotrichum capitatum in a severe aplastic anemia patient. METHODS: Identification of the pathogenic bacteria was done by sequencing and mass spectrometric analysis. RESULTS: The fungal infection was isolated from blood cultures. The pathogenic bacteria were identified as Geotrichum capitatum. The infection was primarily cured by voriconazole and caspofungin monotherapy...
May 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29724307/-effect-of-cd106-mesenchymal-stem-cell-on-bone-marrow-vascular-failure-in-patients-with-aplastic-anemia
#12
Shi Hong Lu, Mei Li Ge, Yi Zhou Zheng, Shao Guang Yang, Fang Chen, Ya Hong You, Zhong Chao Han
Objective To investigate the vascularization ability of mesenchymal stem cells(MSCs)and explore its influencing factors in aplastic anemia(AA) patients. Methods MSCs were isolated from the bone marrow of AA patients(AA MSCs) and normal controls(N MSCs) were cultured and then evaluated by flow cytometry and immunofluorescene staining technique.The expression level of vascular cell adhesion molecule-1(CD106) was detected by gene sequencing,and the content and fluorescene intensity of CD106+ MSCs was determined by fluorescence-activated cell sorting...
April 28, 2018: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/29724291/comparison-of-two-dosages-of-rabbit-antithymocyte-globulin-r-atg-in-treating-children-with-severe-aplastic-anemia
#13
Shao-Fen Lin, Su Liu, Hong-Man Xue, Jun-Bin Huang, Jian Wang, Qi-Hui Chen, Bi-Hong Zhang, Chun Chen
In this study, efficacy and safety of two different dosages of rabbit antithymocyte globulin (r-ATG) combined with cyclosporine (CsA) in treating children with severe aplastic anemia (SAA) were compared. The clinical data of 122 SAA children treated by r-ATG/CsA between Jan 2005 and Jan 2017 at Sun Yat-sen Memorial Hospital of Sun Yat-sen University were retrospectively analyzed. The r-ATG dose of 55 cases was 2.5mg/(kg·d, group 1), and in the other 67 cases it was 3.5 mg/(kg·d, group 2). r-ATG was continuously administered for 5 days...
May 1, 2018: Die Pharmazie
https://www.readbyqxmd.com/read/29722478/successful-reduced-intensity-conditioning-hematopoietic-stem-cell-transplantation-for-paroxysmal-nocturnal-hemoglobinuria-with-aplastic-anemia-in-two-children
#14
Jeffrey R Andolina, Ariel L Reinish, Razia Akhtar, Suzie Noronha, Jessica C Shand, Angela Girvin, David N Korones, Lauren B Bruckner, Craig A Mullen, Kevin J Curran, Farid Boulad
Paroxysmal nocturnal hemoglobinuria (PNH) is an extremely rare cause of bone marrow failure in children. We report two children who presented with pancytopenia, and were diagnosed with PNH with severe aplastic anemia. Both children underwent upfront, successful hematopoietic stem cell transplantation with reduced-intensity conditioning. One patient had a syngeneic donor, and one patient had a 10/10 matched unrelated donor. Neither patient developed graft versus host disease, infections, or recurrent PNH. Reduced-intensity conditioning hematopoietic stem cell transplantation is a reasonable therapy for PNH with marrow failure in children...
May 3, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29720492/sustained-clonal-hematopoiesis-by-hla-lacking-hematopoietic-stem-cells-without-driver-mutations-in-aplastic-anemia
#15
Tatsuya Imi, Takamasa Katagiri, Kazuyoshi Hosomichi, Yoshitaka Zaimoku, Viet Hoang Nguyen, Noriharu Nakagawa, Atsushi Tajima, Tetsuichi Yoshizato, Seishi Ogawa, Shinji Nakao
Clonal hematopoiesis by hematopoietic stem progenitor cells (HSPCs) that lack an HLA class I allele (HLA- HSPCs) is common in patients with acquired aplastic anemia (AA); however, it remains unknown whether the cytotoxic T lymphocyte (CTL) attack that allows for survival of HLA- HSPCs is directed at nonmutated HSPCs or HSPCs with somatic mutations or how escaped HLA- HSPC clones support sustained hematopoiesis. We investigated the presence of somatic mutations in HLA- granulocytes obtained from 15 AA patients in long-term remission (median, 13 years; range, 2-30 years)...
May 8, 2018: Blood Advances
https://www.readbyqxmd.com/read/29713553/acquired-amegakaryocytic-thrombocytopenia-and-pure-red-cell-aplasia-in-thymoma
#16
Sumit Dahal, Eliza Sharma, Suyash Dahal, Binav Shrestha, Bikash Bhattarai
Association of thymoma with myasthenia gravis, pure red cell aplasia, and aplastic anemia is well documented. However, thymoma complicated by acquired amegakaryocytic thrombocytopenia (AAMT) is rarely reported. Here, we present a case of a 60-year-old male with past medical history of recurrent invasive thymoma who presented with cough and blood in sputum. He was found to have severe normocytic normochromic anemia and thrombocytopenia that did not improve with intravenous steroids or multiple transfusions of red cells and platelets...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29712817/-auto-immune-signature-in-aplastic-anemia
#17
EDITORIAL
Antonio M Risitano
No abstract text is available yet for this article.
May 2018: Haematologica
https://www.readbyqxmd.com/read/29710551/saponins-from-panax-notoginseng-leaves-improve-the-symptoms-of-aplastic-anemia-and-aberrant-immunity-in-mice
#18
Yanna Zhao, Xiaohua Sun, Xiaoling Yu, Ruilan Gao, Liming Yin
Aplastic anemia (AA) is usually treated with immunosuppressive agents, but their efficacy and safety are not satisfactory. Panax notoginseng saponins (PNS) promote the proliferation of hematopoietic stem/progenitor cells. This study aimed to examine the effects of leaf PNS (LPNS) on hematopoiesis and T cells in mouse models of AA. The experiments were performed in normal mice and AA mice (controls, cyclosporine, and low, medium, and high doses of LPNS). Hematopoietic cells were counted using colony formation assays...
April 2, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29708883/graves-disease-following-allogenic-hematopoietic-stem-cell-transplantation-for-severe-aplastic-anemia-case-report-and-literature-review
#19
Ahu Paketçi, Korcan Demir, Özlem Tüfekçi, Sezer Acar, Ayhan Abacı, Şebnem Yılmaz, Ece Böber
BACKGROUND: Similar autoimmune processes (defective T-cell function) take place during the pathogenesis of aplastic anemia (AA) and Graves' disease (GD). Antithyroid drugs used for the management of GD may induce AA and GD may occur following treatment of severe aplastic anemia (SAA). CASE PRESENTATION: Clinical and laboratory investigations were performed for an 11-year-and-2-month-old girl who was referred for bilateral exophthalmus and abnormal thyroid function tests...
April 25, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29703819/new-insights-from-studies-of-clonal-hematopoiesis
#20
Christopher J Gibson, David P Steensma
Clonal hematopoiesis (CH) describes an asymptomatic expansion of blood cells descended from a single hematopoietic stem cell. Recent studies have shown that CH increases in frequency with aging, and is often driven by somatic mutations in genes that are recurrently mutated in hematologic malignancies. When CH is associated with a mutation in a leukemia-associated gene at a variant allele frequency of 0.02 or greater, it is termed "clonal hematopoiesis of indeterminate potential" (CHIP).  CHIP has a 0...
April 27, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
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