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Aplastic Anaemia

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...
August 22, 2016: 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%...
June 28, 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
Hua Zhu, Rong Mu Luo, Zuo Luan, Vincent Lee, Yi Ping Zhu, Cheng Juan Luo, Xiang Feng Tang, Ying Jian Si, Jing Chen
Haploidentical haematopoietic stem cell transplantation (haplo-HSCT) used to be a third-line treatment option for childhood severe aplastic anaemia (SAA). We conducted this retrospective study of 36 children (38 transplants) who received haplo-HSCT from human leucocyte antigen (HLA)-mismatched related donors between July 2002 and November 2013 at five HSCT centres in China, including 17 cases that were 5/6 HLA matched (Group 1) and 21 that were 4/6 or 3/6 HLA matched (Group 2). Although patients in Group 2 had a higher incidence of grade II-IV acute graft-versus-host disease (57·9% vs...
September 2016: British Journal of Haematology
W R Yang, L P Jing, K Zhou, G X Peng, Y Li, L Ye, Y Li, J P Li, H H Fan, L Song, X Zhao, Y Yang, F K Zhang, L Zhang
OBJECTIVE: To analyze the clinical characteristics and to evaluate immunosuppressive therapy (IST) response and survival in hepatitis-associated aplastic anemia (HAAA). METHODS: We retrospectively analyzed clinical characteristics, IST response, long-term survival and clonal evolution in 41 HAAA patients, and compared those with age and bone marrow failure matched idiopathic aplastic anemia (IAA) patients. RESULTS: The prevalence of HAAA among cases of SAA was 4...
May 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
J C W Marsh, G J Mufti
Aplastic anaemia (AA) is frequently associated with other disorders of clonal haemopoiesis such as paroxysmal nocturnal haemoglobinuria (PNH), myelodysplastic syndrome (MDS) and T-large granular lymphocytosis. Certain clones may escape the immune attack within the bone marrow environment and proliferate and attain a survival advantage over normal haemopoietic stem cells, such as trisomy 8, loss of heterozygosity of short arm of chromosome 6 and del13q clones. Recently acquired somatic mutations (SM), excluding PNH clones, have been reported in around 20-25 % of patients with AA, which predispose to a higher risk of later malignant transformation to MDS/acute myeloid leukaemia...
August 2016: International Journal of Hematology
Smita Sood
INTRODUCTION: Multidrug-resistant gram-negative bacteria cause infections which are hard to treat and cause high morbidity and mortality. Due to limited therapeutic options there is a renewed interest upon older antimicrobials which had fallen into disuse as a result of toxic side effects. One such antibiotic is chloramphenicol which was sidelined due to reports linking its use with the development of aplastic anaemia. AIM: A study was conducted to evaluate the susceptibility of chloramphenicol in light of the emerging problem of multi-drug resistant gram negative bacteria (MDR GNB)...
February 2016: Journal of Clinical and Diagnostic Research: JCDR
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