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Aplastic Anaemia

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...
January 20, 2017: British Journal of Haematology
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
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
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
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...
October 5, 2016: Journal of Cellular and Molecular Medicine
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
Nicola A Hanania, Phillip Korenblat, Kenneth R Chapman, Eric D Bateman, Petr Kopecky, Pierluigi Paggiaro, Akihito Yokoyama, Julie Olsson, Sarah Gray, Cecile T J Holweg, Mark Eisner, Charles Asare, Saloumeh K Fischer, Kun Peng, Wendy S Putnam, John G Matthews
BACKGROUND: In phase 2 trials, lebrikizumab, an anti-interleukin-13 monoclonal antibody, reduced exacerbation rates and improved FEV1 in patients with uncontrolled asthma, particularly in those with high concentrations of type 2 biomarkers (eg, periostin or blood eosinophils). We undertook replicate phase 3 studies to assess the efficacy and safety of lebrikizumab in patients with uncontrolled asthma despite inhaled corticosteroids and at least one second controller medication. METHODS: Adult patients with uncontrolled asthma, pre-bronchodilator FEV1 40-80% predicted, and stable background therapy were randomly assigned (1:1:1) with an interactive voice-web-based response system to receive lebrikizumab 37·5 mg or 125 mg, or placebo subcutaneously, once every 4 weeks...
October 2016: Lancet Respiratory Medicine
S Nakao, R P Gale
No abstract text is available yet for this article.
September 2, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
D Gómez-Almaguer, A Vázquez-Mellado, J R Navarro-Cabrera, V Abello-Polo, V Milovic, J García, A L Basquiera, S Saba, G Balladares, J Vela-Ojeda, S Gómez, A Karduss-Aurueta, A Bustinza-Álvarez, A Requejo, L Feldman, J C Jaime-Pérez, S Yantorno, G Kusminsky, C H Gutiérrez-Aguirre, J Arbelbide, J Martinez-Rolon, G Jarchum, G Jaimovich, L Riera, E Pedraza-Mesa, L Villamizar-Gómez, M Á Herrera-Rojas, M M Gamboa-Alonso, C Foncuberta, G Rodríguez-González, M A García Ruiz-Esparza, E Hernández-Maldonado, M Paz-Infanzón, E González-López, G J Ruiz-Argüelles
We studied 298 patients with severe aplastic anaemia (SAA) allografted in four Latin American countries. The source of cells was bone marrow (BM) in 94 patients and PBSCs in 204 patients. Engraftment failed in 8.1% of recipients with no difference between BM and PBSCs (P=0.08). Incidence of acute GvHD (aGvHD) for BM and PBSCs was 30% vs 32% (P=0.18), and for grades III-IV was 2.6% vs 11.6% (P=0.01). Chronic GvHD (cGvHD) between BM and PBSCs was 37% vs 59% (P=0.002) and extensive 5% vs 23.6% (P=0.01). OS was 74% vs 76% for BM vs PBSCs (P=0...
January 2017: Bone Marrow Transplantation
Mallikarjuna Shetty, Anukonda Moti Venkata Raja Narendra, Krishna Prasad Adiraju, Nageshwar Rao Modugu
INTRODUCTION: Aplastic Anaemia (AA) is a syndrome characterized by peripheral pancytopenia with hypo-cellular marrow. Acquired idiopathic AA is the most common variety, probably of an autoimmune aetiology. Bone Marrow Transplantation (BMT) is the treatment of choice but cost is the limiting factor. Antithymocyte Globulin and Cyclosporine-A is an alternative to BMT. Cyclosporine alone has been tried as a single agent in resource poor setting. AIM: The study was conducted with the aim to observe the treatment response in aplastic anaemia to Cycloserine-A...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
Lynne Pearce
Essential facts Haematological malignancies are a diverse group of cancers that affect the blood, bone marrow and lymphatic systems. The main categories are lymphoma, leukaemia, myeloma, myelodysplastic syndromes and myeloproliferative neoplasms. In addition, there are subtypes of lymphoma and leukaemia, as well as more rare haematological cancers that have their own categories. There are also borderline conditions such as aplastic anaemia and other non-malignant bone marrow failure syndromes. The charity Bloodwise says 38,000 people in Britain are diagnosed every year with blood cancer or a related disorder, making it the country's fifth most common cancer...
July 27, 2016: Nursing Standard
S Andrade, D B Bartels, R Lange, L Sandford, J Gurwitz
WHAT IS KNOWN AND OBJECTIVE: Metamizole was withdrawn from the market in the United States and several European countries following reports of fatal agranulocytosis among users, but is still available in many countries in Europe, South America and Asia. Over the past several decades, a number of epidemiologic studies have been conducted to quantify the risk of agranulocytosis and other adverse effects associated with metamizole and other non-narcotic analgesics. The objective of this study was to perform a systematic review of the safety of metamizole...
October 2016: Journal of Clinical Pharmacy and Therapeutics
Sneh Singh, Monica Gupta, Gajender Singh, Ashima Batra, Pratibha Dhiman, Abhinav, Sonia Chhabra, Rajeev Sen
Gelatinous bone marrow transformation (GTBM) also known as serous atrophy is a degenerative change in the hematopoietic bone marrow and is a rare well recognized pathological entity. It was earlier described mainly in association with anorexia nervosa and psychiatric eating disorders, but recently it has been reported in ulcerative colitis, tuberculosis, chronic renal diseases, immuno suppressed states (mainly HIV infection), malignancies and Kala azar. Treatment is based on treating the underlying disease...
June 2016: Indian Journal of Hematology & Blood Transfusion
Mukand Lal, Manoj Thakur, Sandeep Kashyap
Avascular necrosis of head of femur is a rare presentation in Dyskeratosis Congenita (DC). Aplastic anaemia, increased susceptibility to infections and fatal pulmonary complications often complicate the clinical scenario in these patients leading to short life span and difficulty in performing surgical interventions. The diagnosis initially and subsequent management by total hip replacement posed a great challenge. To our knowledge this is the first case report of any intervention in form of total hip Arthroplasty done in DC...
June 2016: Indian Journal of Hematology & Blood Transfusion
Ashleigh Scott, Kirk Morris, Jason Butler, Anthony K Mills, Glen A Kennedy
BACKGROUND: plastic anaemia (AA) is a rare acquired bone marrow failure syndrome resulting from immune mediated destruction of haematopoietic stem cells (HSC). For adults in whom first-line haematopoietic progenitor cell transplantation (HPCT) is not feasible, combination anti-thymocyte globulin (ATGAM) plus cyclosporine A (CsA) is standard therapy; however, there is minimal data available regarding the optimal ATGAM dosage in terms of efficacy and survival. AIMS: Our institutions have historically used different dosing protocols of ATGAM in the treatment of AA...
July 12, 2016: Internal Medicine Journal
Diana Averbuch, Dan Engelhard, Anna Pegoraro, Simone Cesaro
Neutropenic patients with malignancies and hematopoietic stem cell transplant recipients are prone to severe infections. Reversal of neutropenia with granulocyte transfusion (GTX) from donors stimulated with GCSF with/without steroids aims to improve outcome of infection. Cochrane analysis of randomized controlled studies, however, failed to show reduced mortality following GTX in conjunction with antibiotics. Non-randomized studies published during the last 20 years produced a very broad spectrum of results...
July 5, 2016: Current Drug Targets
Lan-Ping Xu, Shun-Qing Wang, De-Pei Wu, Jian-Min Wang, Su-Jun Gao, Ming Jiang, Cun-Bang Wang, Xi Zhang, Qi-Fa Liu, Ling-Hui Xia, Xin Wang, Xiao-Jun Huang
We conducted a prospective, multicentre study to confirm the feasibility of haplo-identical transplantation in treatment of severe aplastic anaemia (SAA) as salvage therapy, by analysing the outcomes of 101 patients who received haplo-identical transplantation between June 2012 and October 2015. All cases surviving for more than 28 d achieved donor myeloid engraftment. The median time for myeloid engraftment was 12 (range, 9-25) days and 15 (range, 7-101) days for platelets, with a cumulative platelet engraftment incidence of 94·1 ± 0·1%...
October 2016: British Journal of Haematology
Chizuru Saito, Ken Ishiyama, Hirohito Yamazaki, Yoshitaka Zaimoku, Shinji Nakao
Patients with mild hypomegakaryocytic thrombocytopenia (HMT) that does not meet the diagnostic criteria for a definite disease entity may potentially progress to aplastic anaemia (AA) that is refractory to therapy. To clarify the clinical picture of HMT, we prospectively followed 25 HMT patients with white blood cell count >3·0 × 10(9) /l, haemoglobin level >100 g/l and platelet count of <100·0 × 10(9) /l in the absence of morphological and karyotypic abnormalities in the bone marrow. Glycosylphosphatidylinositol-anchored protein-deficient blood cells [paroxysmal nocturnal haemoglobinuria (PNH)-type cells] were detected in 7 of the 25 (28%) patients and elevated plasma thrombopoietin (TPO, also termed THPO) levels (>320 pg/ml) were observed in 11 (44%) patients...
June 28, 2016: British Journal of Haematology
G Socié, H Schrezenmeier, P Muus, I Lisukov, A Röth, A Kulasekararaj, J W Lee, D Araten, A Hill, R Brodsky, A Urbano-Ispizua, J Szer, A Wilson, P Hillmen
BACKGROUND: Paroxysmal nocturnal haemoglobinuria (PNH) is a rare disease. Although much progress has been made in the understanding of the pathophysiology of the disease, far less is known with respect to the clinical outcomes of patients with PNH. Few retrospective studies provide survival estimates, and even fewer have explored the clinical heterogeneity of the disease. Haemolytic and aplastic anaemia (AA) forms of the disease have been recognised as main disease categories, with the haemolytic form being associated with the worst prognosis by the largest studied cohort some years ago...
September 2016: Internal Medicine Journal
Syed Ghulam Musharraf, Amna Jabbar Siddiqui, Tahir Shamsi, Arshi Naz
Acute leukaemia (AL) is a critical neoplasm of white blood cells. Diagnosing AL requires bone marrow puncture procedure, which many patients do not consent to for it is invasive. Hence sensitive and specific early diagnostic biomarkers are essential for non-invasive diagnosis, new therapeutics and improving the disease prognosis. To differentiate the metabolic alterations associated with acute lymphoblastic leukaemia (ALL) and acute myeloid leukaemia (AML), we investigated serum of ALL and AML patients in comparison with two controls using gas chromatography coupled with triple quadrupole tandem mass spectrometry and multivariate statistical analysis...
June 10, 2016: Hematological Oncology
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