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https://www.readbyqxmd.com/read/28212262/molecular-cytogenetic-approach-to-characterize-novel-and-cryptic-chromosome-abnormalities-in-childhood-myeloid-malignances-of-fanconi-anemia
#1
Maria L R Borges, Roberto R Capela de Matos, Bethânia D A Silva Amaral, Eliane M Soares-Ventura, Edinalva P Leite, Mariluze O D Silva, Maria T M Nogueira Cornélio, Maria L M Silva, Thomas Liehr, Terezinha D J Marques-Salles
Myeloid malignancies can be either primary or secondary, whether or not a specific cause can be determined. Fanconi anemia (FA), a rare constitutional bone marrow failure, usually presents an increased possibility of clonal evolution, due to the increase in chromosomal instability, TP53 activation, and cell death. The evolution of FA may include aplastic anemia by the progressive failure of the bone marrow and myelod neoplasias, such as acute myeloid leukemia and myelodysplastic syndrome. Chromosome abnormalities, particularly of chromosomes, 1, 3, and 7, during the aplastic phase of the disease are predictive of evolution to acute myeloid leukemia/myelodysplastic syndrome...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28211781/post-therapeutic-recovery-of-serum-interleukin-35-level-might-predict-positive-response-to-immunosuppressive-therapy-in-pediatric-aplastic-anemia
#2
Zhen Huang, Hongfei Tong, Yuan Li, Haixia Zhou, Jiangchao Qian, Juxiang Wang, Jichen Ruan
BACKGROUND: The predictive value of interleukin-35 (IL-35) on efficacy of immunosuppressive therapy (IST) in aplastic anemia (AA) has not been well investigated. The aim of the study was to evaluate the association between serum IL-35 level and response to IST in pediatric AA. METHODS: A total of 154 children with AA and 154 controls were included between January 2012 and December 2013. Blood and bone marrow fluid specimens were collected. Serum level of IL-35 was determined by enzyme-linked immunosorbent assay...
February 17, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28195151/in-patients-with-chronic-aplastic-anemia-bone-marrow-derived-mscs-regulate-the-treg-th17-balance-by-influencing-the-notch-rbp-j-foxp3-ror%C3%AE-t-pathway
#3
Hongbo Li, Lin Wang, Yan Pang, Zujun Jiang, Zenghui Liu, Haowen Xiao, Haijia Chen, Xiaohu Ge, Hai Lan, Yang Xiao
The standard treatment for aplastic anemia (AA) in young patients is a matched sibling hematopoietic stem cell transplant. Transfusion of a chronic AA patient with allogeneic bone marrow-derived mesenchymal stromal cells (BMMSCs) is currently being developed as a cell-based therapy, and the safety and efficacy of such transfusions are being continuously improved. Nevertheless, the mechanisms by which BMMSCs exert their therapeutic effects remain to be elucidated. In this study, mesenchymal stromal cells (MSCs) obtained from bone marrow donors were concentrated and intravenously injected into 15 chronic AA patients who had been refractory to prior immunosuppressive therapy...
February 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28194076/outcome-of-cyclosporine-monotherapy-in-patients-of-aplastic-anemia-experience-of-a-tertiary-care-hospital-in-eastern-india
#4
Prakas Kumar Mandal, Suvraneel Baul, Tuphan Kanti Dolai, Rajib De, Prantar Chakrabarti
INTRODUCTION: Immune suppression is a crucial pillar for treatment of aplastic anemia. Cyclosporine monotherapy is an easily available, affordable therapeutic option with good safety profile. METHODS AND MATERIALS: This prospective study was conducted over a period of 2 years from June 2012 to July 2014. The diagnosis and response to treatment of aplastic anemia was established as per published criteria. Follow up was done at 3 and 6 months in order to assess the response...
March 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28178130/de-escalation-empirical-antibiotic-therapy-improved-survival-for-patients-with-severe-aplastic-anemia-treated-with-antithymocyte-globulin
#5
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/28164595/differential-expression-of-cd52-cd14-and-hla-dr-on-cd4-monocytes-in-three-types-of-acquired-bone-marrow-failure-syndromes
#6
Yang Kai, Dai Yuanyuan, Guan Shihe, Mu Xuanxuan, Zhang Hao, Pan Ying, Wu Yuanyuan, Wang Aihua, Sun Beibei, TongYang, Zhou Tingdong
BACKGROUND: Aplastic anemia (AA), paroxysmal nocturnal hemoglobinuria (PNH), and myelodysplastic syndrome (MDS) are the common spectrums of acquired bone marrow failure syndromes (BMFs). Accurate and timely diagnosis is a significant clinical challenge because of the overlapping features. The pathogenesis is not fully understood, but several studies have suggested that defective monocyte functions play an important role. We aimed to find whether the different expressions of CD52, CD14 and HLA-DR on CD4+ monocytes would be helpful in the preliminary diagnosis of acquired BMFs...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28154281/fulminant-laryngeal-tracheobronchial-pulmonary-aspergillosis-a-rare-and-fatal-complication-in-allogeneic-hematopoietic-stem-cell-transplantation-recipients
#7
Tao Tao, Ying-Hui Zhang, Sheng-Li Xue, De-Pei Wu, Feng Chen
A 23-year-old man who had previously undergone allogeneic hematopoietic stem cell transplantation (allo-HSCT) for severe aplastic anemia was diagnosed with invasive laryngeal-tracheobronchial-pulmonary aspergillosis after presenting with a persistent dry cough at six months post-transplantation based on the findings of laryngoscopy and fiberoptic bronchoscopy. A fiberoptic bronchoscope was used to remove the obstructive material from the patient's airway and posaconazole plus caspofungin were administered to successfully to treat the patient...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28154186/structural-analysis-reveals-the-deleterious-effects-of-telomerase-mutations-in-telomerase-associated-bone-marrow-failure-syndromes
#8
Hunter Hoffman, Cory Rice, Emmanuel Skordalakes
Naturally occurring mutations in the ribonucleoprotein reverse transcriptase, telomerase, are associated with the bone marrow failure syndromes dyskeratosis congenita (DKC), aplastic anemia (AA), and idiopathic pulmonary fibrosis (IPF). However, the mechanism by which these mutations impact telomerase function remains unknown. Here we present the structure of the human telomerase c-terminal extension (CTE or thumb domain) determined by the method of single-wavelength anomalous diffraction (SAD) to 2.31 A resolution...
February 1, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28153603/high-programmed-death-1-expression-on-t-cells-in-aplastic-anemia
#9
Wanhong Zhao, Yilin Zhang, Pengyu Zhang, Juan Yang, Longjin Zhang, Aili He, Wanggang Zhang, Tamura Hideto
Programmed death 1 (PD-1) has been reported to be associated with aberrant regulation of T cells activation in aplastic anemia (AA). However, the connection between PD-1 expression status and AA needs to be further explored. The aim of this study is to investigate PD-1 expression status on T cells in AA patients and to explore the effect of PD-1 on apoptosis of T cells and BMHSCs. The concentration of platelet, lymphocyte and hemoglobin in peripheral blood of AA patients and healthy volunteers was detected by automatic blood-counter system...
January 30, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28133157/-a-case-of-eosinophilic-enteritis-needed-to-distinguish-from-a-tumor-of-the-small-intestine
#10
Gen Tsujio, Naoki Aomatsu, En Wang, Yoshihito Yamakoshi, Daisuke Nagashima, Toshiki Hirakawa, Takehiko Iwauchi, Takafumi Nishii, Junya Morimoto, Kazunori Nakazawa, Seika Tei, Yasutake Uchima, Kazuhiro Takeuchi
A 75-year-old woman was diagnosed with aplastic anemia 6 months ago and was under follow-up at our hospital. She had originallypresented to our hospital because of ongoing diarrhea and abdominal pain. Her blood tests showed a rise in inflammatorymarkers (WBC count was 6,900/mL[eosinophil was 1.3%]and CRP was 8.60mg/dL). Her abdominal computed tomography(CT)scan showed gastric wall and small intestine edema as well as ascites. There was no evidence of free air. We diagnosed her with generalized peritonitis and performed an emergencyoperation ...
November 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28129526/addition-of-androgens-improves-survival-in-elderly-patients-with-acute-myeloid-leukemia-a-goelams-study
#11
Arnaud Pigneux, Marie C Béné, Philippe Guardiola, Christian Recher, Jean-Francois Hamel, Mathieu Sauvezie, Jean-Luc Harousseau, Olivier Tournilhac, Francis Witz, Christian Berthou, Martine Escoffre-Barbe, Denis Guyotat, Nathalie Fegueux, Chantal Himberlin, Mathilde Hunault, Martine Delain, Bruno Lioure, Eric Jourdan, Frederic Bauduer, Francois Dreyfus, Jean-Yves Cahn, Jean-Jacques Sotto, Norbert Ifrah
Purpose Elderly patients with acute myeloid leukemia (AML) have a poor prognosis, and innovative maintenance therapy could improve their outcomes. Androgens, used in the treatment of aplastic anemia, have been reported to block proliferation of and initiate differentiation in AML cells. We report the results of a multicenter, phase III, randomized open-label trial exploring the benefit of adding androgens to maintenance therapy in patients 60 years of age or older. Patients and Methods A total of 330 patients with AML de novo or secondary to chemotherapy or radiotherapy were enrolled in the study...
February 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28128288/the-spleen-microenvironment-influences-disease-transformation-in-a-mouse-model-of-kit-d816v-dependent-myeloproliferative-neoplasm
#12
Natalie Pelusi, Maike Kosanke, Tamara Riedt, Corinna Rösseler, Kristin Seré, Jin Li, Ines Gütgemann, Martin Zenke, Viktor Janzen, Hubert Schorle
Activating mutations leading to ligand-independent signaling of the stem cell factor receptor KIT are associated with several hematopoietic malignancies. One of the most common alterations is the D816V mutation. In this study, we characterized mice, which conditionally express the humanized KIT(D816V) receptor in the adult hematopoietic system to determine the pathological consequences of unrestrained KIT signaling during blood cell development. We found that KIT(D816V) mutant animals acquired a myeloproliferative neoplasm similar to polycythemia vera, marked by a massive increase in red blood cells and severe splenomegaly caused by excessive extramedullary erythropoiesis...
January 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28124968/-accurate-diagnosis-of-pseudomonas-luteola-in-routine-microbiology-laboratory-on-the-occasion-of-two-isolates
#13
Muharrem Çiçek, Gülşen Hasçelik, H Kaan Müştak, K Serdar Diker, Burçin Şener
Pseudomonas luteola which was previously known as Chryseomonas luteola; is a gram-negative, non-fermentative, aerobic, motile, non-spore-forming bacillus. It is frequently found as a saprophyte in soil, water and other damp environments and is an opportunistic pathogen in patients with underlying medical disorders or with indwelling catheters. It has been reported as an uncommon cause of bacteremia, sepsis, septic arthritis, meningitis, endocarditis, and peritonitis. Thus, early and accurate identification of this rare species is important for the treatment and also to provide information about the epidemiology of P...
October 2016: Mikrobiyoloji Bülteni
https://www.readbyqxmd.com/read/28121342/clinical-applications-of-haploidentical-hematopoietic-stem-cell-transplantation-in-severe-aplastic-anemia
#14
P Zhang, K Feng, Y Xue, C-X Zhang, Y Wang, X-L Li
OBJECTIVE: The purpose of this study was to investigate the efficacy and safety of haploidentical allogeneic hemopoietic stem cell transplantation (allo-HSCT) in severe aplastic anemia (SAA) and prophylaxis of complications involved. PATIENTS AND METHODS: 8 patients with clinically diagnosed SAA (5 cases of SAA-I and 3 cases of SAA-II) were recruited, with the parents as the donors of hemopoietic stem cells. The conditioning regimen before HSCT included cyclophosphamide, fludarabine, pig anti-human lymphocyte immune globulin (p-ALG) and/or total body irradiation (TBI)...
January 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28115201/life-and-death-of-yeast-telomerase-rna
#15
REVIEW
Yulia Vasianovich, Raymund J Wellinger
Telomerase reverse transcriptase elongates telomeres to overcome their natural attrition and allow unlimited cellular proliferation, a characteristic shared by stem cells as well as the majority of malignant cancerous cells. The telomerase holoenzyme comprises a core RNA molecule, a catalytic protein subunit and other accessory proteins. Malfunction of certain telomerase components can cause serious genetic disorders including dyskeratosis congenita and aplastic anemia. A hierarchy of tightly regulated steps constitute the process of telomerase biogenesis, which if interrupted or misregulated, can impede production of a functional enzyme and severely affect telomere maintenance...
January 20, 2017: Journal of Molecular Biology
https://www.readbyqxmd.com/read/28107815/upfront-haploidentical-transplant-for-acquired-severe-aplastic-anemia-registry-based-comparison-with-matched-related-transplant
#16
Lan-Ping Xu, Song Jin, Shun-Qing Wang, Ling-Hui Xia, Hai Bai, Su-Jun Gao, Qi-Fa Liu, Jian-Min Wang, Xin Wang, Ming Jiang, Xi Zhang, De-Pei Wu, Xiao-Jun Huang
BACKGROUND: Haploidentical donor (HID) hematopoietic stem cell transplantation (HSCT) is an alternative treatment method for severe aplastic anemia (SAA) patients lacking suitable identical donors and those who are refractory to immunosuppressive therapy (IST). The current study evaluated the feasibility of upfront haploidentical HSCT in SAA patients. METHODS: We conducted a multicenter study based on a registry database. One hundred fifty-eight SAA patients who underwent upfront transplantation between June 2012 and September 2015 were enrolled...
January 21, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28100318/-clinical-characteristics-of-clonal-evolution-after-immunosuppressive-therapy-in-children-with-severe-very-severe-aplastic-anemia
#17
Jing-Liao Zhang, Tian-Feng Liu, Li-Xian Chang, Xia Chen, Yuan-Yuan Ren, Cong-Cong Sun, Chao Liu, Wen-Bin An, Yang Wan, Xiao-Juan Chen, Wen-Yu Yang, Shu-Chun Wang, Ye Guo, Yao Zou, Yu-Mei Chen, Xiao-Fan Zhu
OBJECTIVE: To evaluate the clinical characteristics and risk factors of clonal evolution after immunosuppressive therapy (IST) in children with severe/very severe aplastic anemia (SAA/VSAA). METHODS: The clinical data of 231 children with newly-diagnosed SAA/VSAA who received IST were retrospectively studied. The incidence and risk factors of clonal evolution after IST were analyzed. RESULTS: The 5-year overall survival rate of the 231 patients was 82...
January 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28097850/-a-regenerative-anemia-in-infants-2-cases-of-pearson%C3%A2-s-syndrome
#18
José M Martínez de Zabarte Fernández, Carmen Rodríguez-Vigil Iturrate, Cristina Martínez Faci, Inmaculada García Jiménez, Laura Murillo Sanjuan, Ascensión Muñoz Mellado
Anemia is very common in infants. Although its causes are usually not severe and treatable, proper etiologic diagnosis should be established. When anemia is non-regenerative, it can be caused by aplastic anemia, myelodysplastic syndrome, bone marrow infiltration or hematopoietic factors deficiencies. Another possible cause is Pearson's syndrome, a rare mitochondrial disease that causes non-regenerative anemia associated with other cytopenias, pancreatic insufficiency, lactic acidosis and great variability in clinical presentation conditioned by heteroplasmy...
1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28096088/how-i-treat-acquired-aplastic-anemia
#19
Andrea Bacigalupo
Acquired severe aplastic anemia (SAA) is a rare hematologic disease associated with significant morbidity and mortality. Immune destruction of hemopoietic stem cells, plays an important role in the pathogenesis, as shown by successful treatment with immunosuppressive agents (IST), leading to transfusion independence, or complete recovery of peripheral blood counts, in a proportion of patients. Growth factors, can be combined with IST, and may improve response rates, as recently shown with thrombopoietin analogs...
January 17, 2017: Blood
https://www.readbyqxmd.com/read/28090056/suspected-pulmonary-infection-with-trichoderma-longibrachiatum-after-allogeneic-stem-cell-transplantation
#20
Tomoaki Akagi, Chizuko Kawamura, Norio Terasawa, Kohei Yamaguchi, Kohmei Kubo
Aspergillus and Candida species are the main causative agents of invasive fungal infections in immunocompromised human hosts. However, saprophytic fungi are now increasingly being recognized as serious pathogens. Trichoderma longibrachiatum has recently been described as an emerging pathogen in immunocompromised patients. We herein report a case of isolated suspected invasive pulmonary infection with T. longibrachiatum in a 29-year-old man with severe aplastic anemia who underwent allogeneic stem cell transplantation...
2017: Internal Medicine
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