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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/28592049/-the-chinese-guidelines-for-the-diagnosis-and-treatment-of-invasive-fungal-disease-in-patients-with-hematological-disorders-and-cancers-the-fifth-revision
#7
(no author information available yet)
Invasive fungal disease(IFD) is a common yet highly lethal complication in patients with hematological malignancies receiving chemotherapy or stem cell transplantation, as well as immune suppressive conditions including aplastic anemia and other malignancies. According to the diagnostic criteria, patients are defined as proven, probable, possible and undefined IFD based on the evidence provided by histopathologic/cytologic, culture, radiographic and biomarker examinations. For the management of IFD, the major treatment strategies consist of prophylaxis, empirical, diagnostic-driven and target therapy...
June 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28588839/human-embryonic-stem-cell-therapy-for-aplastic-anemia
#8
Geeta Shroff, Rakesh Gupta, Lalduhawmi Zadeng
Human embryonic stem cell (hESC) therapy is the potential therapeutic option for the treatment of patients with aplastic anemia (AA). The study showed a remarkable improvement in the AA patient subsequent to hESC administration. No adverse events occurred in the patient. hESC therapy is safe and effective for AA patients.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28588809/cytopenia-first-hepatitis-second-an-unusual-sequence-in-aplastic-anemia
#9
Ferras Alashkar, Daniel Föhring, Ulrich Dührsen, Hideo Andreas Baba, Alexander Röth
To the best of our knowledge, this is the first report of aplastic anemia (AA) preceding autoantibody-negative autoimmune hepatitis (AIH) with successful treatment of both conditions with the same immunosuppressive regimen, resulting in hematopoietic reconstitution and remission of AIH.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28583279/pet-in-benign-bone-marrow-disorders
#10
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/28574201/standardized-high-sensitivity-flow-cytometry-testing-for-paroxysmal-nocturnal-hemoglobinuria-in-children-with-acquired-bone-marrow-failure-disorders-a-single-center-u-s-study
#11
Rachel E Donohue, Andrea N Marcogliese, Ghadir S Sasa, M Tarek Elghetany, Alka A Redkar, Alison A Bertuch, Choladda V Curry
BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder that has not been well documented in children, particularly those with acquired bone marrow failure disorders (ABMFD) - acquired aplastic anemia (AAA) and myelodysplastic syndrome (MDS). Therefore, we sought to determine the prevalence of PNH populations in children with ABMFD. METHODS: PNH testing was performed in children with an ABMFD diagnosis using high sensitivity (≥0...
June 2, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/28566565/gata-transcription-factors-basic-principles-and-related-human-disorders
#12
Tohru Fujiwara
The development of mature blood cell from hematopoietic stem cells is regulated by transcription factors that coordinate the expression of lineage-specific genes. GATA transcription factors are zinc finger DNA-binding proteins that play crucial roles in various biological processes, including hematopoiesis. Among GATA family proteins, GATA-1, GATA-2, and GATA-3 are essential for hematopoiesis. GATA-1 functions to promote development of erythrocytes, megakaryocytes, eosinophils, and mast cells. Mutations in GATA-1 are associated with acute megakaryoblastic leukemia (AMKL), congenital erythroid hypoplasia (Diamond-Blackfan anemia; DBA), and X-linked anemia and/or thrombocytopenia...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28562512/an-unusual-cause-of-fever-and-rash-in-a-child-with-severe-aplastic-anemia
#13
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
#14
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/28535627/-significance-of-anti-epo-receptor-antibody-in-immune-related-pancytopenia
#15
T Wang, S F Hao, R Fu, H Q Wang, Z H Shao
Objective: To confirm the presence of erythropoietin receptor (EPOR) antibody in patients with immune-related pancytopenia (IRP) and to evaluate the significance of EPOR in IRP. Methods: A total of 59 newly-diagnosed IRP patients, 62 patients with IRP in remission, 14 patients with aplastic anemia (AA), 15 patients with myelodysplastic syndromes (MDS) and 33 healthy controls were enrolled in this study from January 2013 to June 2015 in Tianjin Medical University General Hospital. The anti-EPOR antibody was detected by enzyme-linked immunosorbent assay(ELISA)...
May 16, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28525589/bone-marrow-involvement-in-systemic-lupus-erythematosus
#16
Emilie Chalayer, Nathalie Costedoat-Chalumeau, Odile Beyne-Rauzy, Jacques Ninet, Stephane Durupt, Jacques Tebib, Bouchra Asli, Olivier Lambotte, Martine Ffrench, Christian Vasselon, Pascal Cathébras
BACKGROUND: Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia, and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a target organ in SLE. Our objective was to describe this bone marrow involvement. METHODS: This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome...
May 19, 2017: QJM: Monthly Journal of the Association of Physicians
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
#17
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
#18
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/28504860/allogeneic-bone-marrow-derived-mesenchymal-stromal-cells-expanded-in-vitro-for-treatment-of-aplastic-anemia-a-multicenter-phase-ii-trial
#19
Yan Pang, Hao-Wen Xiao, Hang Zhang, Zeng-Hui Liu, Li Li, Yang Gao, Hong-Bo Li, Zu-Jun Jiang, Huo Tan, Jing-Ren Lin, Xin Du, Jian-Yu Weng, Da-Nian Nie, Dong-Jun Lin, Xiang-Zhong Zhang, Qi-Fa Liu, Duo-Rong Xu, Hai-Jia Chen, Xiao-Hu Ge, Xiao-Yan Wang, Yang Xiao
We conducted a phase II, noncomparative, multicenter study to assess the efficacy and safety of allogeneic bone marrow-derived mesenchymal stromal cells (BM-MSCs) expanded in vitro for patients with aplastic anemia (AA) refractory to immunosuppressive therapy. Seventy-four patients from seven centers received allogeneic BM-MSCs at a dose of 1-2 × 10(6) cells/kg per week for 4 weeks. Responses were assessed at 0.5, 1, 2, 3, 6, 9, and 12 months after the first cells infusion. Patients with response at 1 month continued to receive four infusions...
May 15, 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28499781/aplastic-anemia-and-cytotoxic-t-lymphocyte-antigen-4-haploinsufficiency-treated-with-bone-marrow-transplantation
#20
Payal Makadia, Arvind Srinath, Suneeta Madan-Khetarpal, Marianne McGuire, Elena Infante, Jing Zhang, Raymond E Felgar, Amy W Davis, Hey J Chong, Randy M Windreich
No abstract text is available yet for this article.
May 9, 2017: Journal of Allergy and Clinical Immunology in Practice
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