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Severe aplastic anemia

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https://www.readbyqxmd.com/read/28631177/trail-in-cd8-t-cells-from-patients-with-severe-aplastic-anemia
#1
Chunyan Liu, Mengying Zheng, Tian Zhang, Rong Fu, Huaquan Wang, Ting Wang, Weiwei Qi, Zonghong Shao
Severe aplastic anemia (SAA) is an autoimmune disease caused mainly by activated T lymphocytes. Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is a member of TNF family, which can induce apoptosis and play a significant role in the pathogenesis of many autoimmune disorders. In this study, we sought to investigate the role of TRAIL in peripheral CD8+ T cells (CTLs) from SAA patients to clarify the autoimmune mechanisms of bone marrow failure in SAA. The expression of TRAIL and TRAIL-R2 in CTLs from SAA patients and normal controls were determined by flow cytometry, real-time PCR, and western blot...
June 19, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28630090/t-cell-transcriptomes-from-paroxysmal-nocturnal-hemoglobinuria-patients-reveal-novel-signaling-pathways
#2
Kohei Hosokawa, Sachiko Kajigaya, Keyvan Keyvanfar, Wangmin Qiao, Yanling Xie, Danielle M Townsley, Xingmin Feng, Neal S Young
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder originating from hematopoietic stem cells and is a life-threating disease characterized by intravascular hemolysis, bone marrow (BM) failure, and venous thrombosis. The etiology of PNH is a somatic mutation in the phosphatidylinositol glycan class A gene (PIG-A) on the X chromosome, which blocks synthesis of the glycolipid moiety and causes deficiency in GPI-anchored proteins. PNH is closely related to aplastic anemia, in which T cells mediate destruction of BM...
June 19, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28617164/liver-transplantation-using-graft-from-a-donor-with-aplastic-anemia
#3
Soheila Milani, Mohsen Aliakbarian, Ebrahim Khaleghi
Liver transplantation (LT) is a life-saving procedure in patients with end-stage liver disease. The number of patients in the waiting list for LT has steadily increased over time, so removing liver from deceased donors with unusual disorders could possibly be used for transplantation. We describe a case of LT from a donor with "aplastic anemia" to a patient with liver failure due to hepatitis C. Aplastic anemia is a syndrome of bone marrow failure. Aplastic anemia is one of the most common conditions linked to transfusion dependence, and long-term repeated transfusion inevitably results in iron overload...
June 2017: Progress in Transplantation
https://www.readbyqxmd.com/read/28612279/prevalence-and-clinical-outcomes-of-hepatitis-b-virus-infection-in-patients-with-aplastic-anemia
#4
Pan Zhao, Qing Gao, Qiulian He, Jing Tan
The association of HBV infection with other hematopoietic diseases has been discussed previously. However, the clinical significance and clinical outcomes of HBV infection in AA patients have not been clarified. In this study, we sought to investigate the prevalence and related events of HBV in patients with AA who received immunosuppressive therapy. We retrospectively analyzed 245 patients with acquired AA. The HBsAg positivity rate was 14.69% in this group of AA patients. No significant difference was observed in the severity of AA patients with HBV infection and in those without (P = 0...
June 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28596646/evaluation-of-bone-marrow-microvessel-density-in-patients-with-aplastic-anemia
#5
Venkatesan Somasundaram, Manvir Singh Tevatia, Abhishek Purohit, Ankur Ahuja, Manoranjan Mahapatra, Seema Tyagi, Haraprasad Pati, Renu Saxena
Bone marrow microenvironment plays a crucial role in the growth of hemopoietic cells and bone marrow function, which in turn depends on an intact microvasculature. Our study assesses the microvessel density (MVD) in the bone marrow of aplastic anemia (AA) patients, compares with MVD of controls and MVD among the different types of AA. Bone marrow specimens from 60 patients with AA and 17 controls were studied. There were 33 patients with non severe AA (NSAA), 12 patients with severe AA (SAA) and 15 patients with very severe AA (VSAA)...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28594081/new-evidence-suggesting-a-dissociated-etiology-for-cribra-orbitalia-and-porotic-hyperostosis
#6
Frances Rivera, Marta Mirazón Lahr
OBJECTIVES: Porotic hyperostosis (PH), characterized by porotic lesions on the cranial vault, and cribra orbitalia (CO), a localized appearance of porotic lesions on the roof of the orbits, are relatively common osteological conditions. Their etiology has been the focus of several studies, and an association with anemia has long been suggested. Anemia often causes bone marrow hypertrophy or hyperplasia, leading to the expansion in trabecular or cranial diploic bone as a result of increased hematopoiesis...
June 8, 2017: American Journal of Physical Anthropology
https://www.readbyqxmd.com/read/28583279/pet-in-benign-bone-marrow-disorders
#7
REVIEW
Wouter van der Bruggen, Andor W J M Glaudemans, Edo Vellenga, Riemer H J A Slart
This review aims to describe the current status of benign bone marrow (BM) imaging using PET. BM imaging is important as the BM is not only involved in poiesis of different vital cell lines and can be affected by primary BM disorders, but it is also frequently affected by several extramedullary diseases. Indications for the use of PET in benign BM disorders are the detection of extramedullary hematopoiesis, evaluation of patients with a discrepancy between BM histology and clinical status, visualizing BM infarctions, location of the optimal site for BM biopsy, diagnosing and staging of other hematological BM disorders, evaluation of radiotherapy effect on BM, and evaluation of BM transplantation...
July 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28562512/an-unusual-cause-of-fever-and-rash-in-a-child-with-severe-aplastic-anemia
#8
Elis Yuexian Lee, Wen Quan Derrick Lian, Prasad Iyer, Rajat Bhattacharyya
A 4-year-old girl with severe aplastic anemia and 2 previous failed T-depleted haploidentical peripheral blood stem cell transplants developed persistent neutropenic fever and multiple erythematous maculopapular rashes 2 days after her third T-replete haploidentical bone marrow transplant. Skin biopsy confirmed the diagnosis of Trichosporon asahii infection. She was on caspofungin prophylaxis which is not effective against Trichosporon. A high index of suspicion, prompt investigation, and appropriate treatment with voriconazole for 4 months was instrumental in controlling the infection and she remains well presently 9 months posttransplant with full donor chimerism and free from infection...
May 29, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28554856/beneficial-role-of-low-dose-atg-in-unrelated-stem-cell-transplantation-for-adult-patients-with-acquired-severe-aplastic-anemia-reduction-of-graft-versus-host-disease-gvhd-and-improvement-of-gvhd-free-failure-free-survival-rate
#9
Sung-Soo Park, Dae Hun Kwak, Young-Woo Jeon, Jae-Ho Yoon, Sung-Eun Lee, Byung-Sik Cho, Ki-Seong Eom, Yoo-Jin Kim, Hee-Je Kim, Seok Lee, Chang-Ki Min, Seok-Goo Cho, Dong-Wook Kim, Woo-Sung Min, Jong Wook Lee
Stem cell transplantation from an unrelated donor (URD SCT) is often considered in patients with severe aplastic anemia (SAA) whom immunosuppressive therapy failed and matched sibling donor is not available. To reduce the incidence of graft-versus-host disease (GVHD) in URD SCT, introduction of antithymocyte globulin (ATG) to the conditioning regimen has been proposed. Although ATG was shown to play a role in reducing GVHD in a cohort with diverse hematologic diseases, its role in SAA remains uncertain. The aim of this study was to determine the efficacy and toxicity of ATG in URD SCT for adult patients with SAA...
May 26, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28522577/shorter-leukocyte-telomere-length-is-associated-with-higher-risk-of-infections-a-prospective-study-of-75-309-individuals-from-the-general-population
#10
Jens Helby, Børge G Nordestgaard, Thomas Benfield, Stig E Bojesen
In the general population, older age is associated with short leukocyte telomere length and with high risk of infections. In a recent study of allogeneic hematopoietic cell transplantation for severe aplastic anemia, long donor leukocyte telomere length was associated with improved survival in the recipients. These findings suggest that leukocyte telomere length could possibly be a marker of immune competence. Therefore, we tested the hypothesis that shorter leukocyte telomere length is associated with higher risk of infectious disease hospitalization and infection related death...
May 18, 2017: Haematologica
https://www.readbyqxmd.com/read/28512588/severe-aplastic-anemia-following-parvovirus-b19-associated-acute-hepatitis
#11
Masanori Furukawa, Kosuke Kaji, Hiroyuki Masuda, Kuniaki Ozaki, Shohei Asada, Aritoshi Koizumi, Takuya Kubo, Norihisa Nishimura, Yasuhiko Sawada, Kosuke Takeda, Tsuyoshi Mashitani, Masayuki Kubo, Itsuto Amano, Tomoyuki Ootani, Chiho Ohbayashi, Koji Murata, Tatsuichi Ann, Akira Mitoro, Hitoshi Yoshiji
Human parvovirus (HPV) B19 is linked to a variety of clinical manifestations, such as erythema infectiosum, nonimmune hydrops fetalis, and transient aplastic anemia. Although a few cases have shown HPVB19 infection as a possible causative agent for hepatitis-associated aplastic anemia (HAAA) in immunocompetent patients, most reported cases of HAAA following transient hepatitis did not have delayed remission. Here we report a rare case of severe aplastic anemia following acute hepatitis with prolonged jaundice due to HPVB19 infection in a previously healthy young male...
2017: Case Reports in Hepatology
https://www.readbyqxmd.com/read/28481355/population-pharmacokinetics-of-fludarabine-in-patients-with-aplastic-anemia-and-fanconi-anemia-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#12
E Mohanan, J C Panetta, K M Lakshmi, E S Edison, A Korula, N A Fouzia, A Abraham, A Viswabandya, V Mathews, B George, A Srivastava, P Balasubramanian
Although hematopoietic stem cell transplantation (HSCT) with a conditioning regimen consisting of fludarabine (F-araA) and cyclophosphamide (Cy) is associated with improved outcome in young patients with aplastic anemia (AA) and Fanconi anemia (FA), several factors limit the success of the procedure. We evaluated the population pharmacokinetics (POPPK) of F-araA and its influence on HSCT outcome in patients (n=53) with AA and FA undergoing HSCT. Patients carrying a 5'-UTR polymorphism in NT5E gene (rs2295890 G>C) exhibited significantly lower plasma F-araA clearance compared to those with wild-type genotype (7...
May 8, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28473904/from-chronic-immune-thrombocytopenia-to-severe-aplastic-anemia-recent-insights-into-the-evolution-of-eltrombopag
#13
REVIEW
Harinder Gill, Raymond S M Wong, Yok-Lam Kwong
Thrombopoietin (TPO) is the most potent cytokine stimulating thrombopoiesis. Therapy with exogenous TPO is limited by the formation of antibodies cross-reacting with endogenous TPO. Mimetics of TPO are compounds with no antigenic similarity to TPO. Eltrombopag is an orally-active nonpeptide small molecule that binds to the transmembrane portion of the TPO receptor MPL. Initial trials of eltrombopag have centered on immune thrombocytopenia (ITP), which is due to both increased destruction and decreased production of platelets...
May 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28468095/-the-mechanism-of-bone-marrow-derived-mesenchymal-stem-cells-excessive-senescence-in-severe-aplastic-anemia-mouse-model
#14
Y Q Ou, H Y Liu, W Lu, M J Wen, H Liu
Objective: To explore the mechanism of excessive senescence in bone marrow-derived mesenchymal stem cells (BM-MSC) of mouse model with severe aplastic anemia (SAA) . Methods: 40 BALB/c mice were randomly assigned to two groups of control (n=20) and AA (n=20) . SAA mouse model was induced by intraperitoneal injection with IFN-γ and intragastric infusion with busulfan. BM-MSC were isolated and cultured from bone marrow of SAA and healthy mice. The cell morphology was observed by inverted microscope and cell cytoskeleton was stained by Rhodamine-Phalloidin; The level of proliferation was analyzed by CCK-8 method, and cell cycle was tested by flow cytometry...
April 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28443261/diabetes-mellitus-caused-by-secondary-hemochromatosis-after-multiple-blood-transfusions-in-2-patients-with-severe-aplastic-anemia
#15
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
#16
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/28423296/eltrombopag-added-to-standard-immunosuppression-for-aplastic-anemia
#17
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/28385068/transforming-growth-factor-15-increased-in-severe-aplastic-anemia-patients
#18
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/28376627/severe-acute-hepatitis-and-cold-agglutinin-related-hemolytic-anemia-secondary-to-prime-infection-with-epstein-barr-virus
#19
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/28361276/aplastic-anemia-in-a-lung-adenocarcinoma-patient-receiving-pemetrexed
#20
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
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