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https://www.readbyqxmd.com/read/28443261/diabetes-mellitus-caused-by-secondary-hemochromatosis-after-multiple-blood-transfusions-in-2-patients-with-severe-aplastic-anemia
#1
Hyun Jin Kim, Yoon-Myung Kim, Eungu Kang, Beom Hee Lee, Jin-Ho Choi, Han-Wook Yoo
Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT) at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9...
March 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28436973/a-review-of-hematopoietic-cell-transplantation-in-china-data-and-trends-during-2008-2016
#2
L-P Xu, D-P Wu, M-Z Han, H Huang, Q-F Liu, D-H Liu, Z-M Sun, L-H Xia, J Chen, H-X Wang, C Wang, C-F Li, Y-R Lai, J-M Wang, D-B Zhou, H Chen, Y-P Song, T Liu, K-Y Liu, X-J Huang
Hematopoietic cell transplantation (HCT) activity in China was surveyed to assess its current status. A record number of HCTs (21 884: 16 631 allogeneic (76%) and 5253 autologous (24%)) were reported by 76 centers in China between 1 January 2008 and 30 June 2016. HCT trends included continued growth in transplant activity, a continued rapid increase in haploidentical donors (HID), and slower growth for unrelated donors, matched-related donors (MRD) and cord blood transplantation (CBT). The proportion of HID HCT among allogeneic HCTs increased from 29...
April 24, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28426528/comparative-analysis-of-multicolor-flow-cytometry-and-immunohistochemistry-for-the-detection-of-disseminated-tumor-cells
#3
Eszter Szánthó, Bettina Kárai, Gergely Ivády, Judit Bedekovics, István Szegedi, Miklós Petrás, György Ujj, Anikó Ujfalusi, Csongor Kiss, János Kappelmayer, Zsuzsanna Hevessy
Disseminating cells of a primary solid tumor may represent the origin of metastases and relapses. We aimed at comparing the diagnostic efficacy of multicolor flow cytometry (MFC) and morphology/immunohistochemistry (IHC) in the detection of disseminated tumor cells in the bone marrow (BM) and body fluids of patients with solid tumors, and in pediatric neuroblastoma cases. We investigated 72 samples retrospecively from 50 patients by MFC. Morphology/IHC data were available in 48 cases. In the first cohort, 36 samples derived from 34 patients with various forms of suspected and proven solid tumors and in the second cohort, 36 samples of 16 children with suspected and proven neuroblastoma were analyzed at diagnosis or during follow-up in a 4-color setting by MFC, and the results were compared with those obtained by IHC...
April 19, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28425751/development-of-red-blood-cell-autoantibodies-following-treatment-with-checkpoint-inhibitors-a-new-class-of-anti-neoplastic-immunotherapeutic-agents-associated-with-immune-dysregulation
#4
Laura L Cooling, John Sherbeck, Jonathon C Mowers, Sheri L Hugan
Ipilimumab, nivolumab, and pembrolizumab represent a new class of immunotherapeutic drugs for treating patients with advanced cancer. Known as checkpoint inhibitors, these drugs act to upregulate the cellular and humoral immune response to tumor antigens by inhibiting T-cell autoregulation. As a consequence, they can be associated with immune-related adverse events (irAEs) due to loss of self-tolerance, including rare cases of immune-related cytopenias. We performed a retrospective clinical chart review, including serologic, hematology, and chemistry laboratory results, of two patients who developed red blood cell (RBC) autoantibodies during treatment with a checkpoint inhibitor...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28423296/eltrombopag-added-to-standard-immunosuppression-for-aplastic-anemia
#5
Danielle M Townsley, Phillip Scheinberg, Thomas Winkler, Ronan Desmond, Bogdan Dumitriu, Olga Rios, Barbara Weinstein, Janet Valdez, Jennifer Lotter, Xingmin Feng, Marie Desierto, Harshraj Leuva, Margaret Bevans, Colin Wu, Andre Larochelle, Katherine R Calvo, Cynthia E Dunbar, Neal S Young
Background Acquired aplastic anemia results from immune-mediated destruction of bone marrow. Immunosuppressive therapies are effective, but reduced numbers of residual stem cells may limit their efficacy. In patients with aplastic anemia that was refractory to immunosuppression, eltrombopag, a synthetic thrombopoietin-receptor agonist, led to clinically significant increases in blood counts in almost half the patients. We combined standard immunosuppressive therapy with eltrombopag in previously untreated patients with severe aplastic anemia...
April 20, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28411045/pro-inflammatory-effects-of-the-th1-chemokine-cxcl10-in-acquired-aplastic-anaemia
#6
Junhong Li, Meili Ge, Shihong Lu, Jun Shi, Xingxin Li, Min Wang, Jinbo Huang, Yingqi Shao, Zhendong Huang, Jing Zhang, Neng Nie, Yizhou Zheng
CXCL10/IFN-γ-induced protein 10 (IP-10) and its corresponding receptor CXCR3 have long been considered to be involved in the pathophysiology of type 1 T (Th1) cell-orientated autoimmune diseases. However, the exact role of CXCL10 in the pathogenesis of aplastic anaemia (AA) has not been thoroughly studied. The aim of our study was to evaluate the plasma level of CXCL10 and its effects on CD4(+) T cell differentiation in AA. In our study, we found that an elevated plasma level of CXCL10 was negatively correlated with platelet, absolute neutrophil and reticulocyte counts, while it was positively correlated with the proportion of lymphocytes in white blood cells in AA patients...
April 11, 2017: Cytokine
https://www.readbyqxmd.com/read/28407116/lethal-aplastic-anemia-caused-by-dual-immune-checkpoint-blockade-in-metastatic-melanoma
#7
H Helgadottir, L Kis, P Ljungman, J Larkin, R Kefford, P A Ascierto, J Hansson, G Masucci
No abstract text is available yet for this article.
April 12, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28406545/diagnosing-nocturnal-paroxysmal-hemoglobinuria-a-single-center-4-year-experience
#8
T Mercier, T Devos, M Mukovnikova, N Boeckx
INTRODUCTION: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease and can present as a wide range of signs and symptoms. As such, the indication for diagnostic testing for PNH is not always straightforward. Therefore, we analyzed all first-time samples tested over a 56-month period to determine the clinical settings with a high probability of detecting a PNH clone. METHODS: We retrospectively analyzed 323 first-time PNH flow cytometry tests, including LDH, cytopenias, direct antiglobulin test (DAT), and clinical indication for testing as available at the time of testing...
April 13, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28401099/physicians-preferences-and-perceptions-regarding-donor-selection-in-allogeneic-stem-cell-transplantation-in-korea-when-a-matched-domestic-donor-is-not-available
#9
Min Kyung Shin, Sangjin Shin, Ja Youn Lee, Youngil Koh
BACKGROUND: A number of alternative donor options exist for patients who fail to find domestic HLA-matched donors for allogeneic hematopoietic stem cell transplantation (allo-HSCT). We assessed physicians' perspectives on allo-HSCT donor selection when a matched domestic donor is not available. METHODS: We administered a questionnaire survey to 55 hematologists (response rate: 28%) who attended the annual spring conference of the Korean Society of Haematology in 2015...
March 2017: Blood Research
https://www.readbyqxmd.com/read/28385068/transforming-growth-factor-15-increased-in-severe-aplastic-anemia-patients
#10
Yuanyuan Shao, Honglei Wang, Chunyan Liu, Qiuying Cao, Rong Fu, Huaquan Wang, Ting Wang, Weiwei Qi, Zonghong Shao
OBJECTIVES: The patients with severe aplastic anemia (SAA) usually rely on red cell transfusion which lead to secondary iron overload. Transforming growth differentiation factor-15 (GDF-15) plays an important role in erythropoiesis and iron regulation. In this study, we investigated the level of GDF-15 and other indexes of iron metabolism in SAA patients to explore the correlation with GDF-15 and iron overload in SAA. METHODS: The levels of serum GDF-15, hepcidin (Hepc), and erythropoietin (EPO) were determined by ELISA...
April 6, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28383370/dual-force-vaginoplasty-for-treatment-of-segmental-vaginal-aplasia
#11
Ali M El Saman, Mohamad A Farag, Sherif A Shazly, Mohamed Noor, Mohammed K Ali, Essam R Othman, Mansour Khalifa, Tarek A Farghly, Dina A El Saman
BACKGROUND: Vaginal aplasia occurs in 1 in 5,000-10,000 female live births. In this report, we evaluated a novel dual-force vaginoplasty technique for treatment of 11 patients with segmental vaginal aplasia. TECHNIQUE: The principle of the approach is to thin the atretic part between two counteracting forces. The instrument was inserted laparoscopically into the proximal hematocolpos. Two balloon catheters, one for drainage and one for traction, were threaded over the inserter...
May 2017: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28378905/measurements-of-immature-platelets-with-haematology-analysers-are-of-limited-value-to-separate-immune-thrombocytopenia-from-bone-marrow-failure
#12
Annalina Cybulska, Lisa Meintker, Jürgen Ringwald, Stefan W Krause
Detection of immature platelets in the circulation may help to dissect thrombocytopenia due to platelet destruction from bone marrow failure (BMF). We prospectively tested the predictive value of immature platelets, measured as immature platelet fraction (IPF) on the XE-5000 (Sysmex, Kobe, Japan) or percentage of reticulated platelets (rPT) on the CD Sapphire (Abbott Diagnostics, Santa Clara, CA, USA) to separate immune thrombocytopenia (ITP) from BMF (leukaemia, myelodysplastic syndrome, aplastic anaemia)...
April 5, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28376627/severe-acute-hepatitis-and-cold-agglutinin-related-hemolytic-anemia-secondary-to-prime-infection-with-epstein-barr-virus
#13
Guillermo Ontanilla Clavijo, Julia Praena Segovia, Álvaro Giráldez Gallego, María Elisa Cordero Matía, José Manuel Sousa Martín
Epstein-Barr virus, a member of the Herpesviridae family, is responsible for the infectious mononucleosis clinical syndrome, which mainly includes the pharyngitis, fever, and lymphadenopathy triad after incubation for 30-50 days. The liver is involved in 80-90% of patients in a self-limiting transient manner, with jaundice being much more uncommon (5%). From a hematological standpoint it may manifest aplastic anemia, neutropenia, and thrombocytopenia. We report a case of infectious mononucleosis that included severe acute hepatitis and was associated with severe hemolytic anemia secondary to cold agglutinins...
April 3, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28369969/a-rare-potentially-life-threatening-presentation-of-passenger-lymphocyte-syndrome
#14
Thomas J Gniadek, Andrea M McGonigle, R Sue Shirey, Patricia A Brunker, Michael Streiff, Benjamin Philosophe, Evan M Bloch, Paul M Ness, Karen E King
BACKGROUND: Passenger lymphocyte syndrome occurs when donor lymphocytes are transplanted with a solid organ and produce alloantibodies that react with antigens on the recipient's red blood cells (RBCs). Typically, passenger lymphocyte syndrome presents as immunoglobulin G antibody-mediated, extravascular hemolytic anemia with reticulocytosis. Often, the donor was alloimmunized before transplantation. CASE REPORT: A 34-year-old Group O, D+ man with a negative antibody screen received a liver transplant from a Group O, D- donor...
May 2017: Transfusion
https://www.readbyqxmd.com/read/28361276/aplastic-anemia-in-a-lung-adenocarcinoma-patient-receiving-pemetrexed
#15
Goushi Matama, Takaaki Tokito, Hiroaki Takeoka, Yuki Hiraoka, Norikazu Matsuo, Masayuki Nakamura, Hidenobu Ishii, Takashi Kinoshita, Koichi Azuma, Kazuhiko Yamada, Tomoaki Hoshino
Pemetrexed (PEM) is an antimetabolite drug that interferes with enzymes involved in DNA synthesis and also the folate-dependent metabolic processes necessary for DNA replication and homocysteine homeostasis. Continuation maintenance with PEM after induction therapy with PEM plus cisplatin has been the standard form of first-line chemotherapy for advanced non-squamous non-small cell lung cancer. The regimen has a low incidence of bone marrow suppression, and the incidences of anemia, leukopenia, neutropenia and thrombocytopenia exceeding grade 3 are less than 5%...
March 30, 2017: Investigational New Drugs
https://www.readbyqxmd.com/read/28341733/effect-of-antithymocyte-globulin-source-on-outcomes-of-bone-marrow-transplantation-for-severe-aplastic-anemia
#16
Natasha Kekre, Ying Zhang, Mei-Jie Zhang, Jeanette Carreras, Parvez Ahmed, Paolo Anderlini, Elias Hallack Atta, Mouhab Ayas, Jaap Jan Boelens, Carmem Bonfim, H Joachim Deeg, Neena Kapoor, Jong-Wook Lee, Ryotaro Nakamura, Michael A Pulsipher, Mary Eapen, Joseph H Antin
For treatment of severe aplastic anemia, immunosuppressive therapy with horse antithymocyte globulin results in superior response and survival compared with rabbit antithymocyte globulin. This relative benefit may be different in the setting of transplantation as rabbit antithymocyte globulin results in more profound immunosuppression. We analyzed 833 severe aplastic anemia transplants between 2008 and 2013 using HLA-matched siblings (n=546) or unrelated donors (n=287) who received antithymocyte globulin as part of their conditioning regimen and bone marrow graft...
March 24, 2017: Haematologica
https://www.readbyqxmd.com/read/28333839/evidence-for-increased-response-to-induced-endoplasmic-reticulum-stress-in-myeloid-cells-in-acquired-aplastic-anemia
#17
Alpa Sidhu, Michael U Callaghan, Manisha S Gadgeel, Steven A Buck, Andrew M Fribley, Süreyya Savaşan
Autoimmune response targeting the hematopoietic stem cells highlights the current understanding of acquired aplastic anemia (AAA) pathogenesis. Upregulation of the unfolded protein response is the cell's rejoinder to a variety of stresses, which either result in restoring homeostasis or cell death by increased expression of the transcription factor C/EBP homologous protein. We hypothesized that there is an inherent increased sensitivity to various cellular stressors, including the ones that target endoplasmic reticulum (ER) in AAA leading to a decreased proliferation and potentially contributing to susceptibility to autologous cytotoxicity...
April 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28326326/genetic-analysis-of-two-chicken-infectious-anemia-virus-variants-related-gyrovirus-in-stray-mice-and-dogs-the-first-report-in-china-2015
#18
Lichun Fang, Yang Li, Yixin Wang, Jiayuan Fu, Shuai Cui, Xiaohan Li, Shuang Chang, Peng Zhao
Chicken infectious anemia virus (CIAV) causes acute viral infection in chickens worldwide. It can infect chickens of all ages, but the disease is seen only in young chickens and is characterized by hemorrhagic lesions in the muscles, atrophic changes in the lymphoid organs, aplastic bone marrow, and immunosuppression causing increased mortality. Previous studies have demonstrated that CIAV can be isolated from blood specimens of humans and fecal samples of stray cats. In the present study, two variants of CIAV were isolated from fecal samples of mice (CIAV-Mouse) and stray dogs (CIAV-Dog), respectively...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28321074/epstein-barr-virus-associated-lymphoproliferative-disorder-with-encephalitis-following-anti-thymocyte-globulin-for-aplastic-anemia-resolved-with-rituximab-therapy-a-case-report-and-literature-review
#19
Kiyomi Mashima, Shingo Yano, Hiroki Yokoyama, Takeshi Saito, Tomohito Machishima, Takaki Shimada, Yuichi Yahagi, Shinobu Takahara, Katsuki Sugiyama, Yoji Ogasawara, Jiro Minami, Yutaro Kamiyama, Atsushi Katsube, Kazuhito Suzuki, Sayaka Ohshima, Hisashi Yamada, Noriko Usui, Keisuke Aiba
Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (LPDs) sometimes occur following Anti-thymocyte globulin (ATG) administration for allogenic stem cell transplantation but are rare in aplastic anemia (AA) patients. A 55-year-old woman with AA following ATG developed refractory fever and was diagnosed with EBV-LPD. She was successfully treated with weekly rituximab monotherapy; however, she developed EBV encephalitis. She was admitted to the intensive care unit and finally recovered from unconsciousness...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28303517/vascular-and-perivascular-niches-but-not-the-osteoblastic-niche-are-numerically-restored-following-allogeneic-hematopoietic-stem-cell-transplantation-in-patients-with-aplastic-anemia
#20
Liangliang Wu, Wenjian Mo, Yuping Zhang, Ming Zhou, Yumiao Li, Ruiqing Zhou, Shiling Xu, Shiyi Pan, Hui Deng, Ping Mao, Shunqing Wang
Bone marrow (BM) niches, including the osteoblastic, vascular, and perivascular niches, are numerically impaired in patients with aplastic anemia (AA). It remains unclear whether these niches are numerically restored in AA patients after allogenic hematopoietic stem cell transplantation (allo-HSCT). To investigate changes in BM niches, we monitored 52 patients with AA who had undergone allo-HSCT and performed immunohistochemical studies of BM niches using antibodies against CD34, CD146, and osteopontin. After allo-HSCT, patients with AA exhibited a remarkable increase in the number of cellular elements in the BM niches, including the vascular and perivascular cells...
March 16, 2017: International Journal of Hematology
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