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Severe aplastic anemia

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https://www.readbyqxmd.com/read/28715625/-myelofibrosis-in-a-benzene-exposed-cleaning-worker
#1
Roser Bausà, Lydia Navarro, Imma Cortès-Franch
Long-term exposure to benzene has been associated with several blood malignancies, including aplastic anemia, myeloproliferative neoplasms, and different leukemias. We present a case of primary myelofibrosis in a 59-year-old woman who worked as a cleaner at a car dealership and automobile mechanic shop. For 25 years, she used gasoline as a degreaser and solvent to clean engine parts, floors and work desks on a daily basis. She was referred by her primary care provider to the Occupational Health Unit of Barcelona to assess whether her illness was work-related...
July 2017: Archivos de Prevención de Riesgos Laborales
https://www.readbyqxmd.com/read/28713749/mucormycosis-of-the-frontal-sinus-a-rare-case-report-and-review
#2
Nanda Kishore Sahoo, Vishal Kulkarni, Amit K Bhandari, Arun Kumar
Mucormycosis of the frontal sinus are rarely seen in day to day clinical practice. Although this fungus is commonly found in the environment, the disease is usually prevented by the immune system and is hence rare. Well-recognized risk factors for the disease include diabetes mellitus, leukemia, aplastic anemia, myelodysplastic syndrome, blood dyscrasias, and immunosuppressive therapy in organ transplantation, renal disease, sepsis, and severe burns. The disease is primarily found in those who are immunocompromised, but it may also manifest in immuno competent persons...
January 2017: Annals of Maxillofacial Surgery
https://www.readbyqxmd.com/read/28711931/apc-c-is-essential-for-hematopoiesis-and-impaired-in-aplastic-anemia
#3
Jia Wang, Min-Zhi Yin, Ke-Wen Zhao, Fang Ke, Wen-Jie Jin, Xiao-Lin Guo, Tian-Hui Liu, Xiao-Ye Liu, Hao Gu, Xiao-Min Yu, Zhen Li, Li-Li Mu, Deng-Li Hong, Jing Chen, Guo-Qiang Chen
Anaphase promoting complex/cyclosome (APC/C) is essential for cell cycle progression. Recently, its non-mitotic functions were also reported but less studied in several tissues including hematopoietic cells. Here, we developed an inducible Anapc2 (a core subunit of APC/C) knockout mice. The animals displayed a fatal bone marrow failure within 7 days after knockout induction. Their hematopoietic stem and progenitor cells (HSPCs) demonstrated a sharp decline and could form little colony. Further, the results of BrdU label-retaining cell assay showed that the dormant HPSCs lost rapidly...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28696061/outcomes-and-healthcare-utilization-in-children-and-young-adults-with-aplastic-anemia-a-multiinstitutional-analysis
#4
Ashish Gupta, Pingfu Fu, Hasan Hashem, Anant Vatsayan, Steven Shein, Jignesh Dalal
BACKGROUND: Aplastic anemia is a bone marrow failure syndrome with high mortality affecting children and young adults. Although current treatment guidelines recommend hematopoietic stem cell transplant (HCT) for patients with matched sibling donors, outcomes with alternate donor options have been improving. PROCEDURE: We analyzed a validated multiinstitutional pediatric cohort using one of the largest pediatric and young adult database, the Pediatric Health Information System, for patients diagnosed with aplastic anemia (AA) from 2006 to 2015...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28692030/hla-mismatched-bone-marrow-transplantation-in-severe-aplastic-anemia
#5
L S Bachegowda, M V Shah, L W Veltri, A Tanase, U Popat, P Anderlini, J Chen, G Rondon, R E Champlin, S O Ciurea
No abstract text is available yet for this article.
July 10, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28677684/cotransplantation-of-bone-marrow-derived-mesenchymal-stem-cells-in-haploidentical-hematopoietic-stem-cell-transplantation-in-patients-with-severe-aplastic-anemia-an-interim-summary-for-a-multicenter-phase-ii-trial-results
#6
Z Liu, Y Zhang, H Xiao, Z Yao, H Zhang, Q Liu, B Wu, D Nie, Y Li, Y Pang, Z Fan, L Li, Z Jiang, F Duan, H Li, P Zhang, Y Gao, L Ouyang, C Yue, M Xie, C Shi, Y Xiao, S Wang
This corrects the article DOI: 10.1038/bmt.2016.347.
July 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28675769/outcomes-of-allogeneic-stem-cell-transplantation-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-with-or-without-aplastic-anemia
#7
Sung-Eun Lee, Sung Soo Park, Young-Woo Jeon, Jae-Ho Yoon, Byung-Sik Cho, Ki-Sung Eom, Yoo-Jin Kim, Seok Lee, Chang-Ki Min, Hee-Je Kim, Seok-Goo Cho, Dong-Wook Kim, Woo-Sung Min, Jong Wook Lee
OBJECTIVE: The aim of this study was to evaluate the long-term outcomes of allogeneic stem cell transplantation (SCT) in patients with paroxysmal nocturnal hemoglobinuria (PNH) with or without aplastic anemia (AA). METHOD: A total of 33 patients with PNH clones who underwent allogeneic SCT were analyzed. RESULTS: After a median follow-up of 57 months (range, 6.0-151.3), the 5-year estimated overall survival rate was 87.9 ± 5.7%. Four patients died of transplant-related mortality (TRM)...
July 4, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28653480/eltrombopag-for-secondary-failure-of-platelet-recovery-post-allogeneic-hematopoietic-stem-cell-transplant-in-children
#8
Salah Ali, Adam Gassas, Melanie Kirby-Allen, Joerg Krueger, Muhammad Ali, Tal Schechter
Secondary failure of platelet engraftment occurs in 20% of patients undergoing allogeneic HSCT and is associated with poor outcome. Currently, there are no guidelines for treatment of late thrombocytopenia and platelet transfusion is the mainstay of treatment. Here, we describe the use of Eltrombopag to treat secondary failure of platelet recovery following HSCT in a child with severe aplastic anemia. Eltrombopag resulted in recovery of platelet count with no need for platelet transfusion support with no reported side effects...
June 27, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28637584/decreased-tim-3-expression-of-peripheral-blood-natural-killer-cells-in-patients-with-severe-aplastic-anemia
#9
Tian Zhang, Xin Yuan, Chunyan Liu, Yi Li, Hui Liu, Lijuan Li, Kai Ding, Ting Wang, Honglei Wang, Zonghong Shao, Rong Fu
Severe aplastic anemia (SAA) is an autoimmune disease characterized by severe pancytopenia and bone marrow failure. In our previous studies, we found natural killer (NK) cells were aberrant in SAA patients. T cell immunoglobulin mucin-3 (TIM-3), an important regulator of immunity, is widely detected on NK cells and may contribute as a marker of activation and maturation of NK cells. In this study, we found that SAA untreated patients had lower TIM-3 expression on NK cells and CD56(dim) NK subsets compared with normal controls, and were correlated with the severity of pancytopenia of SAA...
April 24, 2017: Cellular Immunology
https://www.readbyqxmd.com/read/28631177/trail-in-cd8-t-cells-from-patients-with-severe-aplastic-anemia
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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-antithymocyte-globulin-in-unrelated-stem-cell-transplantation-for-adult-patients-with-acquired-severe-aplastic-anemia-reduction-of-graft-versus-host-disease-and-improvement-of-graft-versus-host-disease-free-failure-free-survival
#18
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 (SCT) from an unrelated donor (URD) 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, introducting antithymocyte globulin (ATG) into 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
#19
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
#20
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
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