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https://www.readbyqxmd.com/read/28808516/double-whammy-acute-splenic-sequestration-crisis-in-patient-with-aplastic-crisis-due-to-acute-parvovirus-infection
#1
Parminder S Minhas, Jaspreet K Virdi, Rajeshkumar Patel
Splenic dysfunction is a major feature of sickle cell disease (SCD) and can manifest as acute splenic sequestration crisis (ASSC), which is the earliest life-threatening complication seen in patients with SCD. Aplastic crisis is another potentially deadly complication of sickle cell disease that develops when erythrocyte production temporarily drops. Infection with parvovirus B-19 frequently causes aplastic crises. These two complications are known to be mutually exclusive due to their classic presentation signs and symptoms but there have been few cases where a patient can have concomitant presentation of both phenomena, which can result in a fatal outcome...
July 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28805792/immune-reconstitution-in-patients-with-acquired-severe-aplastic-anemia-after-haploidentical-stem-cell-transplantation
#2
X-Y Pei, X-Y Zhao, L-P Xu, Y Wang, X-H Zhang, Y-J Chang, X-J Huang
Immune recovery (IR) after haploidentical stem cell transplantation (haplo-SCT) in severe aplastic anemia (SAA) patients remains relatively unknown. In this study, we examined immune cell subset counts and immunoglobulins in 81 SAA patients from day 30 to day 365 after haplo-SCT. Simultaneously, we determined which factors influence IR and analyzed the effects of immune cell subsets on transplant outcomes. We found that: (i) The reconstitution of different immune cell subsets occurred at different rates after haplo-SCT...
August 14, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28802588/t-cell-costimulation-blockade-promotes-transplantation-tolerance-in-combination-with-sirolimus-and-post-transplantation-cyclophosphamide-for-haploidentical-transplantation-in-children-with-severe-aplastic-anemia
#3
Sarita Rani Jaiswal, Prakash Bhakuni, Shamsuz Zaman, Satish Bansal, Priyanka Bharadwaj, Sneh Bhargava, Suparno Chakrabarti
We conducted a pilot study employing extended T cell costimulation blockade (COSBL) with Abatacept along with sirolimus and post-transplantation cyclophosphamide (PTCy) in 10 patients (median age 12) with severe aplastic anemia (SAA). Nine patients engrafted in the COSBL group, compared to all 10 patients (median 14 vs 13days) treated on PTCy protocols without abatacept (CONTROL group). The incidence of acute graft-versus-host disease (GVHD) was 10.5% in the COSBL group compared to 50% in the CONTROL group (p=0...
August 9, 2017: Transplant Immunology
https://www.readbyqxmd.com/read/28798776/protective-effects-of-chronic-intermittent-hypobaric-hypoxia-pretreatment-against-aplastic-anemia-through-improving-the-adhesiveness-and-stress-of-mesenchymal-stem-cells-in-rats
#4
Jing Yang, Li Zhang, Handong Wang, Zan Guo, Yixian Liu, Yi Zhang, Chuan Wang, Quanhai Li
Aplastic anemia (AA) is a common malignant blood disease, and chronic intermittent hypobaric hypoxia (CIHH) has a beneficial effect against different diseases. The aim of the present study was to investigate the protective effect of CIHH against AA and underlying mechanisms. 5-Fluorouracil and busulfan treatment induced AA model in rats with reduction of hematological parameters and bone marrow tissue injury and decrease of the colony numbers of progenitor cells. CIHH pretreatment significantly reduced the incidence rate of AA and alleviated above symptoms in AA model...
2017: Stem Cells International
https://www.readbyqxmd.com/read/28797779/long-term-outcomes-of-cord-blood-transplantation-from-an-hla-identical-sibling-for-patients-with-bone-marrow-failure-disorders-a-report-from-eurocord-cord-blood-committee-cbc-ctiwp-and-severe-aplastic-anemia-working-party-saawp-of-the-european-society-of-blood
#5
Simona Pagliuca, Régis Peffault de Latour, Fernanda Volt, Franco Locatelli, Marco Zecca, Jean-Hugues Dalle, Patrizia Comoli, Kim Vettenranta, Miguel Angel Diaz, Or Reuven, Yves Bertrand, Cristina Diaz de Heredia, Arnon Nagler, Ardeshir Ghavamzadeh, Sabina Sufliarska, Sarah Lawson, Chantal Kenzey, Vanderson Rocha, Carlo Dufour, Eliane Gluckman, Jakob Passweg, Annalisa Ruggeri
Cord blood transplantation (CBT) from HLA-identical sibling is an attractive option for patients with bone-marrow-failures syndromes (BMF), due to the low risk of graft-versus-host disease (GVHD) and the absence of risk to the donor. We analyzed outcomes of 117 patients with inherited or acquired BMF, who received CBT from a related HLA-identical donor in EBMT centers, between 1988 and 2014. Ninety-seven patients had inherited and 20 patients acquired BMF. Eighty-two patients received a single cord blood (CB) unit, whereas 35 patients received a combination of CB and bone marrow (BM) cells from the same donor...
August 7, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28791840/the-coexistence-of-autoimmune-rheumatic-diseases-and-thymomas
#6
Ying Jiang, Xiaodan Dai, Liping Duan, Yaou Zhou
BACKGROUND: Autoimmune rheumatic diseases (ARDs), involving immune disturbances resulting from auto-inflammatory mechanisms, are a group of diseases characterized by autoimmunity and autoimmunemediated organ damage. Thymoma, whose mechanism is also associated with immune abnormalities, is the most common neoplasm of the anterior mediastinum. But thymoma with ARDs is relatively less frequent. The clinical characteristics of the coexistence of ARDs and thymomas are still not very clear...
March 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/28783143/unrelated-cord-blood-transplantation-in-aplastic-anemia-is-anti-thymocyte-globulin-indispensable-for-conditioning
#7
K Kudo, H Muramatsu, A Narita, N Yoshida, R Kobayashi, H Yabe, M Endo, M Inoue, J Hara, S Kounami, J Inagaki, Y Hashii, K Kato, K Tabuchi, S Kojima
No abstract text is available yet for this article.
August 7, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28777856/-clinical-and-laboratory-features-of-13-cases-of-myeloid-neoplasms-with-double-del-20q
#8
Shuxiao Bai, Chunxiao Wu, Jun Zhang, Suning Chen, Jinlan Pan
OBJECTIVE: To report on clinical and laboratory features of myeloid neoplasms with double del(20q). METHODS: Cytogenetic examination of bone marrow was performed on 13 cases of myeloid neophasms with double del(20q) after 24 hours of cell culture. R-banding was used to analyze the karyotypes. Interphase fluorescence in situ hybridization (FISH) was performed using dual-color probes for 20q11/20q12. RESULTS: Double del(20q) was found to be the sole abnormality in 9 cases, double del(20q) and trisomy 9 was found in 1 case, trisomy del(20q) was found in 1 case, and sole del(20q) clone and double del(20q) clone were found to coexist in 2 cases...
August 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28765987/anastomosis-of-the-external-carotid-artery-and-the-v3-segment-of-the-vertebral-artery-presumed-persistent-second-cervical-intersegmental-artery-diagnosed-by-ct-angiography
#9
Akira Uchino, Naoko Saito, Hiroki Kurita
Congenital anastomosis of the external carotid (ECA) and vertebral (VA) arteries is extremely rare. We report a case of right ECA-VA anastomosis diagnosed by computed tomography (CT) angiography. The presumed dilated ascending pharyngeal artery coursed posteriorly and entered the C1 right transverse foramen. The proximal right VA was aplastic, and the C2 right transverse foramen was absent. Based on these findings, we diagnosed congenital anastomosis of the ECA and V3 segment of the VA, which we presumed to represent persistence of the second cervical intersegmental artery...
August 1, 2017: Surgical and Radiologic Anatomy: SRA
https://www.readbyqxmd.com/read/28764810/cd106-is-a-novel-mediator-of-bone-marrow-mesenchymal-stem-cells-via-nf-%C3%AE%C2%BAb-in-the-bone-marrow-failure-of-acquired-aplastic-anemia
#10
Shihong Lu, Meili Ge, Yizhou Zheng, Jianping Li, Xiaoming Feng, Sizhou Feng, Jinbo Huang, Ying Feng, Donglin Yang, Jun Shi, Fang Chen, Zhongchao Han
BACKGROUND: Acquired aplastic anemia (AA) is characterized by deficiency or dysfunction of the bone marrow (BM) microenvironment. However, little is known about the impairment of BM-derived mesenchymal stem cells (MSCs) in AA patients. METHODS: We used Illumina HiSeqTM 2000 sequencing, quantitative real-time polymerase chain reaction (qRT-PCR), flow cytometry (FCM), and Western blotting to test the expression of CD106 gene (vascular cell adhesion molecule 1 (VCAM1)) and CD106 protein of BM-MSCs...
August 1, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28756883/caspofungin-treatment-for-pulmonary-invasive-fungal-disease-in-hematology-patients-a-retrospective-study-in-a-clinical-practice-setting-in-china
#11
Xiaohui Zhang, Jiong Hu, Yu Hu, He Huang, Jie Jin, Juan Li, Qifa Liu, Zonghong Shao, Jianxiang Wang, Quanshun Wang, Depei Wu, Xiaojun Huang
PURPOSE: Invasive fungal disease (IFD) is a serious complication in patients with hematologic malignancies. Caspofungin is the first approved inhibitor of fungal β-1,3-glucan synthesis. The aim of the present study was to evaluate the effectiveness of caspofungin in the treatment of IFD in patients with hematologic malignancies. METHODS: In this retrospective study, data from the electronic medical records of 1118 inpatients who were admitted to 10 hospitals in China between 2013 and 2014 were analyzed...
July 28, 2017: Clinical Therapeutics
https://www.readbyqxmd.com/read/28751565/incidence-and-outcome-of-acquired-aplastic-anemia-real-world-data-from-patients-diagnosed-in-sweden-from-2000-2011
#12
Krista Vaht, Magnus Göransson, Kristina Carlson, Cecilia Isaksson, Stig Lenhoff, Anna Sandstedt, Bertil Uggla, Jacek Winiarski, Per Ljungman, Mats Brune, Per-Ola Andersson
Aplastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70-80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000-2011...
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28729300/rapamycin-is-highly-effective-in-murine-models-of-immune-mediated-bone-marrow-failure
#13
Xingmin Feng, Zenghua Lin, Wanling Sun, Maile K Hollinger, Marie J Desierto, Keyvan Keyvanfar, Daniela Malide, Pawel Muranski, Jichun Chen, Neal S Young
Acquired aplastic anemia, the prototypical bone marrow failure disease, is characterized by pancytopenia and marrow hypoplasia. Most aplastic anemia patients respond to immunosuppressive therapy, usually as anti-thymocyte globulin and cyclosporine, but some relapse on cyclosporine withdrawal or require long-term administration of cyclosporine to maintain blood counts. In the current study, we tested efficacy of rapamycin as a new or alternative treatment in mouse models of immune-mediated bone marrow failure...
July 20, 2017: Haematologica
https://www.readbyqxmd.com/read/28728504/the-diverse-expression-of-the-wt1-gene-in-patients-with-acquired-bone-marrow-failure-syndromes
#14
Yahong You, Jiali Huo, Shihong Lu, Yingqi Shao, Meili Ge, Jun Shi, Xingxin Li, Jinbo Huang, Zhendong Huang, Jing Zhang, Min Wang, Neng Nie, Yizhou Zheng
Acquired bone marrow failure syndromes (aBMFS) encompass a wide range of diseases. A study to investigate WT1 expression in BM was conducted in 387 patients with aBMFS in China. The WT1 level in patients with aplastic anemia (AA) was significantly lower than that in patients with paroxysmal nocturnal hemoglobinuria (PNH, p = .023) and myelodysplastic syndrome (MDS, p < .001). In addition, the WT1 level in patients with MDS significantly increased as the disease progressed to an advanced stage. Patients with hypoplastic MDS had a differentiated expression level of WT1 compared with that of NSAA (p < ...
July 21, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28728262/-evaluation-of-efficacy-of-immunosuppressive-therapy-plus-recombinant-human-thrombopoietin-for-children-with-severe-aplastic-anemia
#15
K Zhou, C X Liu, Y Li, J P Li, H H Fan, L Zhang, L P Jing, G X Peng, L Ye, Y Li, L Song, X Zhao, W R Yang, Z J Wu, F Chen, F K Zhang
Objective: To evaluate the therapeutic efficacy and safety of immunosuppressive therapy (IST) combined with recombinant human thrombopoietin (rhTPO) for severe aplastic anemia (SAA) in pediatric patients. Method: A retrospective case-control study was conducted and the clinical data of 45 pediatric patients with de novo SAA admitted to the Anemia Diagnosis and Treatment Center of Chinese Academy of Medical Sciences & Blood Disease Hospital during the period from December 2009 to December 2014 were analyzed...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28715625/-myelofibrosis-in-a-benzene-exposed-cleaning-worker
#16
Roser Bausà, Lydia Navarro, Imma Cortès-Franch
Long-term exposure to benzene has been associated with several blood malignancies, including aplastic anemia, myeloproliferative neoplasms, and different leukemias. We present a case of primary myelofibrosis in a 59-year-old woman who worked as a cleaner at a car dealership and automobile mechanic shop. For 25 years, she used gasoline as a degreaser and solvent to clean engine parts, floors and work desks on a daily basis. She was referred by her primary care provider to the Occupational Health Unit of Barcelona to assess whether her illness was work-related...
July 2017: Archivos de Prevención de Riesgos Laborales
https://www.readbyqxmd.com/read/28713749/mucormycosis-of-the-frontal-sinus-a-rare-case-report-and-review
#17
Nanda Kishore Sahoo, Vishal Kulkarni, Amit K Bhandari, Arun Kumar
Mucormycosis of the frontal sinus are rarely seen in day to day clinical practice. Although this fungus is commonly found in the environment, the disease is usually prevented by the immune system and is hence rare. Well-recognized risk factors for the disease include diabetes mellitus, leukemia, aplastic anemia, myelodysplastic syndrome, blood dyscrasias, and immunosuppressive therapy in organ transplantation, renal disease, sepsis, and severe burns. The disease is primarily found in those who are immunocompromised, but it may also manifest in immuno competent persons...
January 2017: Annals of Maxillofacial Surgery
https://www.readbyqxmd.com/read/28711931/apc-c-is-essential-for-hematopoiesis-and-impaired-in-aplastic-anemia
#18
Jia Wang, Min-Zhi Yin, Ke-Wen Zhao, Fang Ke, Wen-Jie Jin, Xiao-Lin Guo, Tian-Hui Liu, Xiao-Ye Liu, Hao Gu, Xiao-Min Yu, Zhen Li, Li-Li Mu, Deng-Li Hong, Jing Chen, Guo-Qiang Chen
Anaphase promoting complex/cyclosome (APC/C) is essential for cell cycle progression. Recently, its non-mitotic functions were also reported but less studied in several tissues including hematopoietic cells. Here, we developed an inducible Anapc2 (a core subunit of APC/C) knockout mice. The animals displayed a fatal bone marrow failure within 7 days after knockout induction. Their hematopoietic stem and progenitor cells (HSPCs) demonstrated a sharp decline and could form little colony. Further, the results of BrdU label-retaining cell assay showed that the dormant HPSCs lost rapidly...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28706392/acquired-aplastic-anemia-associated-with-trisomy-eight-converting-into-acute-myeloid-leukemia
#19
Sumit Grover, Amit Kumar Dhiman, Bhavna Garg, Neena Sood, Vikram Narang
Aplastic anemia (AA) is nowadays considered to be a clonal disorder arising from a defective hematopoietic stem cell developing after a generalized insult to bone marrow. Immunosuppressive treatment (IST) of AA causes suppression of the target dominant population of haematopoietic cells allowing the defective non targeted clones to expand. This may give rise to acute leukemia. Cytogenetic studies for chromosomal aberrations such as trisomy and monosomy may help in detecting such conversions. We present a case of acquired AA in a 60-year-old male presenting with pancytopenia and hypoplastic marrow treated with antithymocyte globulin, converting into myelodysplastic syndrome and later on acute promyelocytic leukemia after being in remission for 4 years...
July 2017: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/28705902/long-term-risk-of-cancer-development-in-adult-patients-with-idiopathic-aplastic-anemia-after-treatment-with-anti-thymocyte-globulin
#20
Joost Gk van der Hem, Liesbeth C de Wreede, Anneke Brand, Hendrik Veelken, Jh Frederik Falkenburg, Constantijn Jm Halkes
No abstract text is available yet for this article.
July 13, 2017: Haematologica
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