keyword
MENU ▼
Read by QxMD icon Read
search

Aplastic

keyword
https://www.readbyqxmd.com/read/28653480/eltrombopag-for-secondary-failure-of-platelet-recovery-post-allogeneic-hematopoietic-stem-cell-transplant-in-children
#1
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/28643364/development-of-clinical-paroxysmal-nocturnal-haemoglobinuria-in-children-with-aplastic-anaemia
#2
Atsushi Narita, Hideki Muramatsu, Yusuke Okuno, Yuko Sekiya, Kyogo Suzuki, Motoharu Hamada, Shinsuke Kataoka, Daisuke Ichikawa, Rieko Taniguchi, Norihiro Murakami, Daiei Kojima, Eri Nishikawa, Nozomu Kawashima, Nobuhiro Nishio, Asahito Hama, Yoshiyuki Takahashi, Seiji Kojima
The clinical significance of paroxysmal nocturnal haemoglobinuria (PNH) in children with aplastic anaemia (AA) remains unclear. We retrospectively studied 57 children with AA between 1992 and 2010. During the follow-up, five patients developed clinical PNH, in whom somatic PIGA mutations were detected by targeted sequencing. The 10-year probability of clinical PNH development was 10·2% (95% confidence interval, 3·6-20·7%). Furthermore, the detection of minor PNH clones by flow cytometry at AA diagnosis was a risk factor for the subsequent development of clinical PNH...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28642072/recombinant-human-thrombopoietin-promotes-platelet-engraftment-and-improves-prognosis-of-patients-with-myelodysplastic-syndromes-and-aplastic-anemia-after-allogeneic-hematopoietic-stem-cell-transplantation
#3
Hong Wang, Man Huang, Ying Zhao, Jia-Qian Qi, Chun Chen, Ya-Qiong Tang, Hui-Ying Qiu, Cheng-Cheng Fu, Xiao-Wen Tang, De-Pei Wu, Chang-Geng Ruan, Yue Han
Poor platelet graft function (PPGF) is a significant complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). However, no optimal treatment has been recommended. This study investigated aspects of platelet recovery after allo-HSCT, including prognostic value and the effect of recombinant human thrombopoietin (rhTPO). We retrospectively analyzed 275 patients who received allo-HSCT in our center. Of them, 135 (49.1%) patients had good platelet graft function (GPGF) and 140 (50.9%) had PPGF...
June 19, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28641647/-clinical-significance-of-serum-thrombopoietin-levels-in-patients-with-aplastic-anaemia
#4
Hai Cheng, Jiang Cao, Wei Chen, Kun-Ming Qi, Zhi-Ling Yan, Kai-Lin Xu
OBJECTIVE: To evaluate the clinical significance of thrombopoietin (TPO) level in diagnosis of pateints with aplastic anaemia (AA). METHODS: The TPO levels in sera from 54 AA patients and 119 healthy controls were examined. A total of 92 samples were collected from AA patients including 43 samples harvested at diagnosis, 23 samples in the cytopenic period after treatment, and 26 samples of patients in partial (n=10) or complete remission (n=16) following immunosuppressive treatment...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28637584/decreased-tim-3-expression-of-peripheral-blood-natural-killer-cells-in-patients-with-severe-aplastic-anemia
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
(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
#13
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
#14
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
#15
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/28583062/multiple-ligament-reconstruction-for-absent-cruciate-and-a-congenital-short-femur-case-report-and-review-of-the-literature
#16
Daniel Kaplan, Taylor Jazrawi, Jason Capo, Eric Strauss
Congenital short femur syndrome is a rare condition affecting about 0.017 of every 1,000 births. Patients frequently have ligamentous abnormalities, most frequently aplastic or hypoplastic cruciate ligaments. Advances in limb lengthening procedures have increased movement expectations for these patients, thus necessitating surgical treatment for their ligamentous abnormalities. An individual case is presented, including history, physical exam, and imaging demonstrating both the short femur and absent cruciate ligaments...
April 2017: Bulletin of the Hospital for Joint Diseases
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
#17
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/28571175/unilateral-absence-of-ethmoid-sinus-and-nasal-turbinates-a-rare-case-report
#18
Vinod Felix, Narendrakumar Veerasigamani
A variety of anatomical variation of paranasal sinus and nasal turbinates exist, as its development is a complex and long standing process. Computerized Tomography (CT) of the paranasal sinuses is a very valuable tool in diagnosing these variations. Preoperatively defining the anatomical variations of the intranasal structures is essential in performing the safe functional endoscopic sinus surgery and to avoid unnecessary complications. Several degrees and combinations of aplasias and hypoplasias have been reported...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28566939/variation-of-a-persistent-primitive-hypoglossal-artery-ppha-as-incidental-finding-in-the-diagnostic-clarification-of-cerebral-vasculopathy-associated-with-intracranial-vasculitis
#19
S Hopf-Jensen, L Marques, M Preiß, W Börm, S Müller-Hülsbeck
We present a very rare variation of a persistent primitive hypoglossal artery (PPHA) arising from the internal carotid artery, detected during a diagnostic angiography. A 50-year-old female patient was admitted with an atypical intracranial hematoma in the left frontal lobe. Catheter angiography revealed intracranial vasculopathy with segmental stenoses, a small aneurysm of the right internal carotid artery bifurcation and a "string of beads" appearance of the left carotid artery, consistent with fibromuscular disease...
June 2017: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/28566565/gata-transcription-factors-basic-principles-and-related-human-disorders
#20
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
keyword
keyword
11337
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"