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Bone marrow dysfunction

Ahmad Kotb, Antonina Klippert, Maria Daskalaki, Ulrike Sauermann, Christiane Stahl-Hennig, Berit Neumann
Granzyme B-expressing (GrB(+)) B cells are thought to contribute to immune dysfunctions in HIV patients, but so far their exact role is unknown. This report demonstrates for the first time the existence of GrB(+) B cells in SIV-infected rhesus macaques, which represent the most commonly used nonhuman primate model for HIV research. Similar to HIV patients, we found significantly higher frequencies of these cells in the blood of chronically SIV-infected rhesus monkeys compared with uninfected healthy ones. These frequencies correlated with plasma viral load and inversely with absolute CD4 T-cell counts...
October 25, 2016: Immunology and Cell Biology
Malorie Simons, Emmanuel Apor, James N Butera, Diana O Treaba
TAFRO syndrome is a rare constellation of symptoms: thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction, and organomegaly. Its pathogenesis involves an excessive and inappropriate cytokine storm, most notably from IL-6, causing multiorgan failure; however, its etiology is undetermined. Starting in 2012, TAFRO syndrome was first identified in Japan as an atypical variant of Castleman's disease. Previous reports include various different treatment protocols with inconsistent survival outcomes...
2016: Case Reports in Hematology
Min-Min Shi, Yuan Kong, Yang Song, Yu-Qian Sun, Yu Wang, Xiao-Hui Zhang, Lan-Ping Xu, Kai-Yan Liu, Xiao-Jun Huang
Poor graft function (PGF) is a serious complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Murine studies suggest that endothelial progenitor cells (EPCs) are preferential supporting cells for hematopoietic stem cells (HSCs) in the bone marrow (BM) microenvironment. Our previous work found that a reduced number of BM EPCs was an independent risk factor for the occurrence of PGF after allo-HSCT. However, little is known about the functional role of BM EPCs and how to improve impaired BM EPCs in PGF...
October 21, 2016: Blood
Animesh Pardanani
: Disease overview:Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extra-cutaneous organs. DIAGNOSIS: The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC expression of CD25 and/or CD2, and presence of KITD816V. Risk stratification: The 2008 World Health Organization (WHO) classification of SM has been shown to be prognostically relevant...
November 2016: American Journal of Hematology
Nick Davis-Poynter, Joseph Yunis, Helen E Farrell
Virus homologues of seven-transmembrane receptors (7TMR) are encoded by all beta- and gammaherpesviruses, suggesting important functional roles. M78 of mouse cytomegalovirus (MCMV) is representative of a family of 7TMR conserved in all betaherpesviruses. M78 family members have been found to exhibit cell-type specific effects upon virus replication in tissue culture and to affect virus pathogenesis in vivo. We reported previously that M78, for which no ligands are known, undergoes rapid, constitutive endocytosis...
2016: PloS One
Nurin Jivani, Carmen Torrado-Jule, Sarah Vaiselbuh, Eleny Romanos-Sirakis
Shwachman-Diamond syndrome (SDS) is an autosomal recessive bone marrow failure syndrome typically characterized by neutropenia and pancreatic dysfunction, although phenotypic presentations vary, and the endocrine phenotype is not well-described. We report a unique case of a patient with SDS who initially presented with hypoglycemia and micropenis in the newborn period and was diagnosed with congenital hypopituitarism. We are not aware of any other cases of SDS documented with this combination of complex endocrinopathies...
October 18, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
Wook-Jin Chung
Pulmonary arterial hypertension (PAH) is a life-threatening disorder with a poor prognosis and causes pulmonary vascular remodeling accompanied with increased pulmonary arterial medial wall thickness and fibrosis, which leads to vascular and right ventricular (RV) dysfunction. Despite treatment with prostacyclin, endothelin antagonist, and phosphodiesterase-5 inhibitors the 1-year mortality rate of PAH still remains high. Recent registries, clinical trials, and basic researches have been increasing the knowledge of PAH and it would contribute to potential therapeutic strategies and better clinical outcome...
September 2016: Journal of Hypertension
Mohan Raizada
Hypertension (HTN) is the most prevalent modifiable risk for cardiovascular disease (CVD) and disorders directly influencing CVD (i.e. obesity, diabetes, chronic kidney disease, obstructive sleep apnea, etc.). About one billion people worldwide have HTN, with American adults having 90% lifetime risk of developing HTN. Despite aggressive campaign for lifestyle changes and advances in drug therapy, HTN remains an immense health, emotional, and economic challenge. This is due, in part, to the fact that ∼50% of HTN patients' blood pressure remains uncontrolled and ∼20% of HTN patients are resistant to or require > antihypertensive drugs...
September 2016: Journal of Hypertension
Ruixue Miao, Zhiling Wang, Qin Guo, Yang Wen, Qiong Liao, Yu Zhu, Min Shu, Chaomin Wan
BACKGROUND: Visceral Leishmaniasis (VL) is a life-threatening parasitic infection transmitted by phlebotomine sandflies. We undertook this study to analyze the clinical features of pediatric VL in a population of Chinese children. METHODS: A retrospective study was performed with pediatric patients (≤14years) diagnosed with VL based on bone-marrow biopsy, serology and diagnosis based on clinical manifestation and the improvement after the experimental drug when negative bone marrow and serology results were shown...
September 30, 2016: Pediatric Infectious Disease Journal
Imranul Alam, Amie K McQueen, Dena Acton, Austin M Reilly, Rita L Gerard-O'Riley, Dana K Oakes, Charishma Kasipathi, Abigail Huffer, Weston B Wright, Michael J Econs
Autosomal dominant osteopetrosis type II (ADO2) is a heritable osteosclerotic bone disorder due to dysfunctional osteoclast activity. ADO2 is caused by missense mutations in the chloride channel 7 (CLCN7) gene characterized by osteosclerosis with multiple fractures. ADO2 can result in osteomyelitis, visual loss and bone marrow failure. Currently, there is no cure for ADO2, and until recently no appropriate animal model of ADO2 existed to understand better the pathogenesis of this disease and to test new therapies...
October 14, 2016: Bone
Ramona Sturm, David Heftrig, Katharina Mörs, Nils Wagner, Kerstin Kontradowitz, Katrin Jurida, Ingo Marzi, Borna Relja
OBJECTIVE: Phagocytizing leukocytes (granulocytes and monocytes) play a fundamental role in immunological defense against pathogens and clearance of cellular debris after tissue injury due to trauma. According to the "two-hit hypothesis", phagocytes become primed due to/after trauma. Subsequently, a secondary stimulus may lead to their exaggerated response. This immune dysfunction can result in serious infectious complications, also depending on trauma injury pattern. Here, we investigated the phagocytizing capacity of leukocytes, and its correlation to trauma injury pattern...
September 29, 2016: Immunobiology
Yuka Suzuki, Saeko Tada-Oikawa, Yasuhiko Hayashi, Kiyora Izuoka, Misa Kataoka, Shunsuke Ichikawa, Wenting Wu, Cai Zong, Gaku Ichihara, Sahoko Ichihara
BACKGROUND: The use of carbon nanotubes has increased lately. However, the cardiovascular effect of exposure to carbon nanotubes remains elusive. The present study investigated the effects of pulmonary exposure to single-walled carbon nanotubes (SWCNTs) and double-walled carbon nanotubes (DWCNTs) on atherosclerogenesis using normal human aortic endothelial cells (HAECs) and apolipoprotein E-deficient (ApoE(-/-)) mice, a model of human atherosclerosis. METHODS: HAECs were cultured and exposed to SWCNTs or DWCNTs for 16 h...
October 13, 2016: Particle and Fibre Toxicology
Falguni Parikh
Febrile patient with thrombocytopenia is commonly encountered by physicians especially during monsoon and perimonsoon period. Infections with protozoa, bacteria and viruses can cause thrombocytopenia with or without disseminated intravascular coagulation. Commonly dengue, malaria, scrub typhus and other rickettsial infections, meningococci, leptospira and certain viral infections present as fever with thrombocytopenia. Occasionally these patients can go on to develop a stormy course with multiorgan dysfunction requiring intensive care unit admission associated with high morbidity and mortality...
February 2016: Journal of the Association of Physicians of India
Praveen Mannam, Navin Rauniyar, TuKiet T Lam, Ruiyan Luo, Patty J Lee, Anup Srivastava
Cigarette smoking is the primary risk factor for COPD which is characterized by excessive inflammation and airflow obstruction of the lung. While inflammation is causally related to initiation and progression of COPD, the mitochondrial mechanisms that underlie the associated inflammatory responses are poorly understood. In this context, we have studied the role played by Mitogen activated protein (MAP) kinase kinase 3 (MKK3), a dual-specificity protein kinase, in cigarette smoke induced-inflammation and mitochondrial dysfunction...
October 4, 2016: Free Radical Biology & Medicine
Metta Dülk, Gyöngyi Kudlik, Anna Fekete, Dávid Ernszt, Krisztián Kvell, Judit E Pongrácz, Balázs L Merő, Bálint Szeder, László Radnai, Miklós Geiszt, Dalma E Csécsy, Tamás Kovács, Ferenc Uher, Árpád Lányi, Virag Vas, László Buday
The commitment steps of mesenchymal stromal cells (MSCs) to adipogenic and other lineages have been widely studied but not fully understood. Therefore, it is critical to understand which molecules contribute to the conversion of stem cells into differentiated cells. The scaffold protein Tks4 plays a role in podosome formation, EGFR signaling and ROS production. Dysfunction of Tks4 causes a hereditary disease called Frank-ter Haar syndrome with a variety of defects concerning certain mesenchymal tissues (bone, fat and cartilage) throughout embryogenic and postnatal development...
October 6, 2016: Scientific Reports
Hongbo Li, Lin Wang, Zujun Jiang, Li Li, Zhifang Xiao, Zenghui Liu, Shuang Zhang, Hui Jin, Lei Su, Yang Xiao
The aim of the present study was to investigate the effect of persistent low-dose iridium-192 (Ir192) exposure on immunological function, chromosome aberration and the telomerase activity of bone marrow mononuclear cells (BMNCs), in order to increase clinical knowledge of the late effects of persistent low-dose Ir192 gamma-ray exposure. Patients (n=54) accidentally exposed to persistent low-dose Ir192 were included in this 10-year follow-up study. Clinical symptoms, peripheral blood, bone marrow, cellular and humoral immune status, chromosome aberrations and the telomerase activity of BMNCs were analyzed in this study...
October 2016: Experimental and Therapeutic Medicine
Darlene Vigil, Nikifor K Konstantinov, Marc Barry, Antonia M Harford, Karen S Servilla, Young Ho Kim, Yijuan Sun, Kavitha Ganta, Antonios H Tzamaloukas
Nephropathy secondary to BK virus, a member of the Papoviridae family of viruses, has been recognized for some time as an important cause of allograft dysfunction in renal transplant recipients. In recent times, BK nephropathy (BKN) of the native kidneys has being increasingly recognized as a cause of chronic kidney disease in patients with solid organ transplants, bone marrow transplants and in patients with other clinical entities associated with immunosuppression. In such patients renal dysfunction is often attributed to other factors including nephrotoxicity of medications used to prevent rejection of the transplanted organs...
September 24, 2016: World Journal of Transplantation
Lei Wang, Xue-Chen Zhao, Wei Cui, Yong-Qiang Ma, Hua-Liang Ren, Xin Zhou, John Fassett, Yan-Zong Yang, Yingjie Chen, Yun-Long Xia, Jie Du, Huihua Li
BACKGROUND: -The recruitment of leukocytes to the vascular wall is a key step in hypertension development. Chemokine receptor CXCR2 mediates inflammatory cell chemotaxis in several diseases, however, the role of CXCR2 in hypertension development and the underlying mechanisms remain unknown. METHODS: -Angiotensin II (490 ng·kg(-1)·min(-1)) or DOCA-salt-induced mouse hypertensive models in genetic ablation, pharmacological inhibition of CXCR2 and adoptive bone marrow transfer mice were used to determine the role of CXCR2 in hypertension (measured by radiotelemetry and tail-cuff system), inflammation (verified by flow cytometry and quantitative real-time PCR analysis), vascular remodeling (studied by haematoxylin and eosin (H&E) and Masson's trichrome staining), vascular dysfunction (assessed by aortic ring), and oxidative stress (indicated by NADPH oxidase activity, DHE staining and quantitative real-time PCR analysis)...
September 27, 2016: Circulation
Hong Seok Kim, Sina Tavakoli, Leigh Ann Piefer, Huynh Nga Nguyen, Reto Asmis
Diabetes promotes the S-glutathionylation, inactivation and subsequent degradation of mitogen-activated protein kinase phosphatase 1 (MKP-1) in blood monocytes, and hematopoietic MKP-1-deficiency in atherosclerosis-prone mice accelerates atherosclerotic lesion formation, but the underlying mechanisms were not known. Our aim was to determine the mechanisms through which MKP-1 deficiency in monocytes and macrophages promotes atherogenesis. Transplantation of MKP-1-deficient bone marrow into LDL-R(-/-) (MKP-1LeuKO) mice accelerated high-fat diet (HFD)-induced atherosclerotic lesion formation...
September 27, 2016: Scientific Reports
Aziz İnan Çelik, Ali Deniz, Mustafa Tangalay, Muhammet Buğra Karaaslan, Emine Bağır Kılıç
Eosinophilic myocarditis (EM) is a rare form of myocarditis that usually presents with heart failure due to eosinophilic infiltration. EM is often a component of hypereosinophilic syndrome (HES). HES is a rare disorder characterized by persistent, marked eosinophilia combined with organ system dysfunction. A 38-year-old man was admitted to emergency services with left inguinal pain and fever, and was hospitalized with diagnosis of nephrolithiasis and urinary tract infection. Intravenous antibiotic therapy of 3 grams meropenem per day and analgesic of 50 mg pethidine per day were administered...
September 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
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