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Aplastic Anaemia

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https://www.readbyqxmd.com/read/28831064/expression-of-cxcr4-on-t-cell-subsets-and-plasma-il-17-concentrations-in-patients-with-aplastic-anaemia
#1
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
#2
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...
July 12, 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
#3
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
#4
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/28641647/-clinical-significance-of-serum-thrombopoietin-levels-in-patients-with-aplastic-anaemia
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/28178130/de-escalation-empirical-antibiotic-therapy-improved-survival-for-patients-with-severe-aplastic-anemia-treated-with-antithymocyte-globulin
#12
COMPARATIVE STUDY
Rong Fu, Tong Chen, Jia Song, Guojin Wang, Lijuan Li, Erbao Ruan, Hui Liu, Yihao Wang, Huaquan Wang, Limin Xing, Yuhong Wu, Hong Liu, Wen Qu, Zonghong Shao
We aimed to investigate the efficacy and safety of de-escalation empirical therapy for controlling infection in patients with severe aplastic anaemia (SAA) treated with antithymocyte globulin (ATG). Eighty-seven ATG-treated SAA patients who had microbiological culture-confirmed infections from 2006 to 2015 in our center were retrospectively analyzed. The efficacy of de-escalation and non-de-escalation therapy was compared. Among all 87 patients, 63 patients were treated with de-escalation therapy and 24 patients with non-de-escalation therapy...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28169418/allogeneic-transplantation-using-cd34-selected-peripheral-blood-progenitor-cells-combined-with-non-mobilized-donor-t%C3%A2-cells-for-refractory-severe-aplastic-anaemia
#13
Enkhtsetseg Purev, Xin Tian, Georg Aue, Jeremy Pantin, Phuong Vo, Reem Shalabi, Robert N Reger, Lisa Cook, Catalina Ramos, Elena Cho, Tat'yana Worthy, Hanh Khuu, David Stroncek, Neal S Young, Richard W Childs
Allogeneic haematopoietic stem cell transplantation is curative for severe aplastic anaemia (SAA) unresponsive to immunosuppressive therapy. To reduce chronic graft-versus-host disease (GVHD), which occurs more frequently after peripheral blood stem cell (PBSC) transplantation compared to bone-marrow transplantation (BMT), and to prevent graft rejection, we developed a novel partial T-cell depleted transplant that infuses high numbers of granulocyte colony-stimulating factor-mobilized CD34(+) selected PBSCs combined with a BMT-equivalent dose of non-mobilized donor T-cells...
March 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28162059/fanconi-anaemia
#14
Doreen Crawford, Annette Dearmun
Fanconi anaemia is a rare inherited genetic condition that can lead to aplastic anaemia and bone marrow failure. People with the condition have a predisposition to some cancers. The condition is named after Swiss paediatrician Guido Fanconi.
February 6, 2017: Nursing Children and Young People
https://www.readbyqxmd.com/read/28107566/recent-advances-in-understanding-clonal-haematopoiesis-in-aplastic-anaemia
#15
REVIEW
Natasha Stanley, Timothy S Olson, Daria V Babushok
Acquired aplastic anaemia (AA) is an immune-mediated bone marrow failure disorder inextricably linked to clonal haematopoiesis. The majority of AA patients have somatic mutations and/or structural chromosomal abnormalities detected as early as at diagnosis. In contrast to other conditions linked to clonal haematopoiesis, the clonal signature of AA reflects its immune pathophysiology. The most common alterations are clonal expansions of cells lacking glycophosphotidylinositol-anchored proteins, loss of human leucocyte antigen alleles, and mutations in BCOR/BCORL1, ASXL1 and DNMT3A...
May 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/27927707/pancytopenia-associated-with-levetiracetam-in-an-epileptic-woman
#16
Rafael García Carretero, Marta Romero Brugera, Monica Olid-Velilla, Inmaculada Salamanca-Ramirez
Haematological toxicity due to antiepileptic drugs is uncommon, but the increased risk of aplastic anaemia has been reported. Few case reports have been published regarding pancytopenia associated with levetiracetam treatment, and its intrinsic pathogenesis is still unknown. We describe the case of a woman aged 77 years who presented with abdominal pain and loss of appetite. She had been taking valproic acid, due to a previous episode of epileptic seizures, and presented with drowsiness and dizziness. Valproate was discontinued and therapy with levetiracetam was initiated...
December 7, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27810336/clinical-scale-expansion-of-cd34-cord-blood-cells-amplifies-committed-progenitors-and-rapid-scid-repopulation-cells
#17
Alba Casamayor-Genescà, Arnau Pla, Irene Oliver-Vila, Noèlia Pujals-Fonts, Sílvia Marín-Gallén, Marta Caminal, Irma Pujol-Autonell, Jorge Carrascal, Marta Vives-Pi, Joan Garcia, Joaquim Vives
Umbilical cord blood (UCB) transplantation is associated with long periods of aplastic anaemia. This undesirable situation is due to the low cell dose available per unit of UCB and the immaturity of its progenitors. To overcome this, we present a cell culture strategy aimed at the expansion of the CD34(+) population and the generation of granulocyte lineage-committed progenitors. Two culture products were produced after either 6 or 14days of in vitro expansion, and their characteristics compared to non-expanded UCB CD34(+) controls in terms of phenotype, colony-forming activity and multilineage repopulation potential in NOD-scid IL2Rγ(null) mice...
March 25, 2017: New Biotechnology
https://www.readbyqxmd.com/read/27800200/successful-resolution-of-preretinal-haemorrhage-with-intravitreal-ranibizumab
#18
Baharuddin Noorlaila, Embong Zunaina, Mohd-Noor Raja-Azmi
We would like to report two cases of preretinal haemorrhage from two different aetiology courses of bleeding being treated with intravitreal ranibizumab and its outcome. Our first case was a 39-year-old man with a diagnosis of severe aplastic anaemia that presented with bilateral premacular haemorrhages in both eyes. His right eye vision was 6/45 and it was counting finger in the left eye. He was treated with intravitreal ranibizumab once to the right eye and twice to the left eye. Right eye showed complete resolution of premacular haemorrhage and minimal residual premacular haemorrhage in the left eye at 3 months after initial presentation...
2016: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/27704726/sera-of-patients-with-spontaneous-tumour-regression-and-elevated-anti-ca-i-autoantibodies-change-the-gene-expression-of-ecm-proteins
#19
Jan Lakota, Radivojka Vulic, Maria Dubrovcakova, Silvia Tyciakova
Spontaneous tumour regression after high-dose therapy and autologous stem cell transplantation is associated with the aplastic anaemia-like syndrome and the presence of polyclonal autoantibodies against carbonic anhydrase I (CA I). When tumour cells were grown in vitro in the presence of patients' sera positive for anti-CA I autoantibodies, their morphological pattern was altered. These changes were accompanied by modifications in the gene expression profile. We observed downregulation of genes of the basal lamina assembly (collagen type IV alpha 4, the laminin subunit gamma 2), the extracellular matrix (collagen type I alpha 1), the cytoskeleton (keratin 14 type I), the collagen triple helix repeat containing 1 and the proto-oncogene WNT7B...
March 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/27660553/comparison-of-a-therapeutic-only-versus-prophylactic-platelet-transfusion-policy-for-people-with-congenital-or-acquired-bone-marrow-failure-disorders
#20
Asma Ashraf, Andreas V Hadjinicolaou, Carolyn Doree, Sally Hopewell, Marialena Trivella, Lise J Estcourt
This is the protocol for a review and there is no abstract. The objectives are as follows: To compare a therapeutic-only versus prophylactic platelet transfusion policy for people with myelodysplasia, inherited or acquired aplastic anaemia, and other congenital bone marrow failure disorders.
September 1, 2016: Cochrane Database of Systematic Reviews
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