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Aplastic Anaemia

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https://www.readbyqxmd.com/read/29334367/unmanipulated-haploidentical-transplantation-conditioning-with-busulfan-cyclophosphamide-and-anti-thymoglobulin-for-adult-severe-aplastic-anaemia
#1
L-P Xu, Z-L Xu, F-R Wang, X-D Mo, T-T Han, W Han, Y-H Chen, Y-Y Zhang, J-Z Wang, Y Wang, C-H Yan, Y-Q Sun, F-F Tang, X-H Zhang, X-J Huang
We conducted a retrospective analysis to evaluate outcomes of haploidentical transplantation in adult severe aplastic anaemia (SAA) patients. Fifty-one adults received haploidentical transplantation between May 2011 and December 2016. Patients were administered busulfan (Bu), cyclophosphamide (Cy) and anti-thymoglobulin (ATG) as conditioning regimens, followed by bone marrow and peripheral blood transplantation. The patients' median age was 25 years. Forty-nine patients survived for more than 28 days and all achieved donor myeloid engraftment...
January 15, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29307285/incidence-of-fanconi-anaemia-in-phenotypically-normal-aplastic-anaemia-patients-in-west-bengal
#2
Atreyee Dutta, Rajib De, Tuphan Kanti Dolai, Pritha Pal, Shanoli Ghosh, Pradip Kumar Mitra, Ajanta Halder
OBJECTIVES: Fanconi anaemia (FA) is a rare inherited bone marrow failure and autosomal recessive blood disorder. FA patients have a higher risk of cancer, including acute myeloid leukaemia and squamous cell carcinoma. Maximum, but not all, affected individuals have one or more somatic abnormalities, including skin, skeletal, genitourinary, gastrointestinal, cardiac and neurological anomalies, etc. Positive stress cytogenetics has immense implications for the treatment and management of FA...
January 7, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29303047/eltrombopag-in-the-management-of-aplastic-anaemia-real-world-experience-in-a-non-trial-setting
#3
Yu-Yan Hwang, Harinder Gill, Thomas S Y Chan, Garret M K Leung, Carol Y M Cheung, Yok-Lam Kwong
OBJECTIVE: The thrombopoietin mimetic eltrombopag has been used in clinical trials for the frontline and salvage treatment of aplastic anaemia (AA). Eltrombopag was investigated in AA patients on a non-trial all-comer basis. METHODS: Consecutive newly diagnosed and relapsed/refractory AA patients were treated with eltrombopag. RESULTS: In a 4.5-year period, 20 consecutive AA patients (newly diagnosed, N = 10; relapsed/refractory, N = 10) at a median age of 47 (22-84) years were treated with eltrombopag...
January 5, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29237659/long-term-follow-up-of-hepatitis-associated-aplastic-anaemia
#4
Cristina Gonçalves, Sandra Ferreira, Susana Nobre, Isabel Gonçalves
Prognosis of hepatitis-associated aplastic anaemia (HAAA) was improved with haematopoietic stem cell transplantation (HSCT) and immunosuppression, but the long-term outcome remains undefined. Case 1: a girl aged 3 years with acute liver failure (ALF) submitted to orthotopic liver transplantation (OLT) subsequently developed aplastic anaemia and HSCT from a compatible sibling was performed. Post-HSCT, the patient developed post-transplant lymphoproliferative disorder and rituximab was administered with good response...
December 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29207763/carbamazepine-induced-thrombocytopenia
#5
Ruhi Kumar, Sridhar Chivukula, Goutham Reddy Katukuri, U K Chandrasekhar, K N Shivashankar
Antiepileptic Drugs (AEDs) are commonly associated with haematological disorders, including anaemia, thrombocytopenia, neutropenia and even bone marrow failure. Fatal disorders like aplastic anaemia are uncommon. On exploring through the literature, older AEDs are more associated with haematological alterations than newer AEDs, and careful monitoring is warranted especially with phenytoin, carbamazepine and valproate. The exact cause of these alterations is not established, though immune mechanisms and pharmacology of individual drugs are the proposed mechanisms, a further research along this path is underway...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29112326/real-world-challenges-and-unmet-needs-in-the-diagnosis-and-treatment-of-suspected-invasive-pulmonary-aspergillosis-in-patients-with-hematological-diseases-an-illustrative-case-study
#6
Martin Hoenigl, Juergen Prattes, Peter Neumeister, Albert Wölfler, Robert Krause
Recent years have seen important advances in the diagnosis of invasive pulmonary aspergillosis (IPA), complemented by the introduction of new therapies. Despite this, IPA remains a major cause of infection-related mortality in patients with hematological diseases. There are two main reasons for this. First, diagnosis of IPA remains a challenge, since risk factors and the clinical, radiological and mycological presentations vary not only by fungal disease stage, but also by patient group (e.g. neutropenic versus non-neutropenic patients)...
November 7, 2017: Mycoses
https://www.readbyqxmd.com/read/29100905/post-transplant-cyclophosphamide-ptcy-as-sole-gvhd-prophylaxis-is-feasible-in-patients-undergoing-peripheral-blood-stem-cell-transplantation-for-severe-aplastic-anaemia-using-matched-sibling-donors
#7
Biju George, Nisham Pn, Anup J Devasia, Uday Kulkarni, Anu Korula, Kavitha M Lakshmi, Aby Abraham, Alok Srivastava, Vikram Mathews
High-dose cyclophosphamide (PTCY) following allogeneic hematopoietic cell transplantation (HSCT) has been shown to be effective in preventing graft-versus-host disease (GVHD) following HLA-matched bone marrow transplantation. We performed a phase II study of PTCY given at 50 mg/kg IV on days 3 and 4 as sole GVHD prophylaxis following HSCT for severe aplastic anemia (SAA) receiving G-CSF-mobilized peripheral blood stem cell (PBSC) grafts from HLA-matched related donors following conditioning with fludarabine, cyclophosphamide and single dose Total Body Irradiation (TBI)...
October 31, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28974509/resolution-of-acute-hepatitis-b-associated-aplastic-anaemia-with-antiviral-therapy
#8
Nicholas Hendren, Joseph Moore, Sandra Hofmann, Siayareh Rambally
A previously healthy 44-year-old woman presented with 3 days of worsening petechial rash, epistaxis and fatigue. Admission labs revealed pancytopenia, low reticulocyte index and elevated liver enzymes. Bone marrow biopsy demonstrated a profoundly hypocellular bone marrow without dysplasia and additional testing demonstrated an acute hepatitis B infection. In the context of an acute hepatitis B infection, elevated liver enzymes and aplastic anaemia, our patient was diagnosed with severe hepatitis-associated aplastic anaemia due to an acute hepatitis B infection...
October 3, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28936381/anal-canal-gas-gangrene-in-aplastic-anaemia-rare-yet-lethal-entity-a-caveat
#9
Sundaramurthi Sudharsanan, Chellappa Vijayakumar, Kumar Manish, T P Elamurugan, Ali S Manwar
Gas gangrene is one of the most serious infections of traumatic and surgical wounds. The importance lies in the fact that, if not managed at the right time, the condition is fatal. Though perianal cellulitis and gangrene are commonly reported in immunocompromised patients, we report the case of a patient with gas gangrene involving only the anal canal extending to the rectum, a rare presentation to be reported in literature.  An 18-year-old lady, a patient of aplastic anaemia - immunodeficiency, developed gas gangrene of the anal canal possibly due to faecal contamination of anal fissures...
July 13, 2017: Curēus
https://www.readbyqxmd.com/read/28831064/expression-of-cxcr4-on-t-cell-subsets-and-plasma-il-17-concentrations-in-patients-with-aplastic-anaemia
#10
Qian Niu, Qiang Zhou, Yumei Liu, Hong Jiang
Acquired aplastic anaemia (AA) is caused by T-cells migrating to and attacking bone marrow (BM) in response to chemokines (e.g., CXCR4). We investigated CXCR4 expressions on circulating T-cell subsets, plasma IL-17A concentrations, and their correlations with AA manifestations. We enrolled 71 patients with acquired AA (36 severe AA cases [SAA] and 35 non-severe AA cases [NSAA]) and 42 healthy volunteers. We used flow cytometry and ELISA to measure circulating CD4(+) and CD8(+) T-cells, their CXCR4 expressions, and plasma IL-17A concentrations...
August 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28699658/telomere-length-and-somatic-mutations-in-correlation-with-response-to-immunosuppressive-treatment-in-aplastic-anaemia
#11
Hee S Park, Si N Park, Kyongok Im, Sung-Min Kim, Jung-Ah Kim, Sang M Hwang, Dong S Lee
We investigated the frequencies of cytogenetic aberrations and somatic mutations of prognostic relevance in 393 patients with aplastic anaemia (AA). Clonality was determined by G-banding/fluorescence in situ hybridization (FISH) (n = 245), and targeted capture sequencing was performed for 88 haematopoiesis-related genes (n = 70). The telomere length (TL) of bone marrow nucleated cells was measured at the single cell level by FISH (n = 135). Eighteen (4·6%) patients showed disease progression, and monosomy 7 (50·0%) was the most predominant cytogenetic evolution at disease transformation...
August 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28666497/paroxysmal-nocturnal-haemoglobinuria-type-iii-presenting-as-portal-and-mesenteric-vein-thrombosis-in-a-young-girl
#12
Shahzad Sarwar, Monazza Chaudhry, Natasha Ali
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired, life-threatening haematological disorder. It is characterised by complement induced haemolytic anaemia, thrombosis and impaired bone marrow function. Thrombosis most commonly occurs in the hepatic, portal, superior mesenteric and cerebral veins. A22-year female, previously diagnosed with severe aplastic anaemia treated with anti-lymphocyte globulin (ALG) and cyclosporine, had become transfusion independent for more than 10 years. She presented with abdominal pain and vomiting, initially diagnosed with portal and superior mesenteric vein thrombosis...
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28643364/development-of-clinical-paroxysmal-nocturnal-haemoglobinuria-in-children-with-aplastic-anaemia
#13
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...
September 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28641647/-clinical-significance-of-serum-thrombopoietin-levels-in-patients-with-aplastic-anaemia
#14
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/28411045/pro-inflammatory-effects-of-the-th1-chemokine-cxcl10-in-acquired-aplastic-anaemia
#15
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/28378905/measurements-of-immature-platelets-with-haematology-analysers-are-of-limited-value-to-separate-immune-thrombocytopenia-from-bone-marrow-failure
#16
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)...
May 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28275823/beyond-immune-thrombocytopenia-the-evolving-role-of-thrombopoietin-receptor-agonists
#17
REVIEW
Francesco Rodeghiero, Giuseppe Carli
Since its discovery, the thrombopoietin (TPO) pathway has been an important pharmaceutical target for the treatment of thrombocytopenia. The first generation of TPO mimetics included peptide agents sharing homology with endogenous TPO, but these introduced a risk of antibody formation to endogenous TPO and were not successful. However, second-generation TPO mimetics or TPO receptor agonists (RAs) are currently being used to treat thrombocytopenia associated with a number of conditions, such as immune thrombocytopenia (ITP), severe aplastic anaemia (SAA), and hepatitis C virus-associated chronic liver disease...
September 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28275389/cd19-cd56-myeloma-arising-in-a-patient-who-failed-two-courses-of-immunosupressive-therapy-for-aplastic-anaemia
#18
Nigel P Murray, M Amparo Ruiz, G Maximiliano Miranda
Patients diagnosed with severe aplastic anaemia and without a compatible bone marrow transplant donor are treated with immunosuppressive therapy. These patients are found with time to develop a clonal disease such as myelodysplasia or paroxysmal nocturnal haemoglobinuria. However, the development of plasma cell dyscrasias is rare. We report the case here of a patient treated with immunosuppressive therapy who went on to develop myeloma 11 months after being diagnosed with severe aplastic anaemia. We include here a review of the literature...
2017: Ecancermedicalscience
https://www.readbyqxmd.com/read/28254831/false-homozygous-hla-genotyping-results-due-to-copy-number-neutral-loss-of-heterozygosity-in-acquired-aplastic-anaemia
#19
Bert Heyrman, Ann De Becker, Sonja Verheyden, Christian Demanet
The aim of this case report is to draw attention on possible false human leucocyte antigen (HLA) genotyping in acquired aplastic anaemia prior to allogeneic haematopoietic stem cell transplantation. In acquired aplastic anaemia loss of heterozygosity (LOH) of chromosome 6p is known to occur in around 12%. We report false HLA genotyping results due to LOH and a coinciding steep rise in neutrophils following filgrastim stimulation in a patient with very severe aplastic anaemia. At diagnosis we obtained heterozygous results on peripheral blood...
March 2, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28195548/herbal-approach-in-the-treatment-of-pancytopenia
#20
REVIEW
Siddhi Manohar Bagwe, Pravin Popatrao Kale, Lokesh Kumar Bhatt, Kedar S Prabhavalkar
Pancytopenia is a health condition in which there is a reduction in the amount of leucocytes, erythrocytes and thrombocytes. If more than one of the blood cells is low then the condition is called as bicytopenia. The pancytopenic condition is observed in treatment of diseased conditions like thalassemia and hepatitis C. Iatrogenically pancytopenia is caused by some antibiotics and anti-HCV drugs. Medical conditions like aplastic anaemia, lymphoma, copper deficiency, and so forth can also cause pancytopenia...
March 1, 2017: Journal of Complementary & Integrative Medicine
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