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thrombotic microangiopathies

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https://www.readbyqxmd.com/read/27919500/favorable-outcome-of-interferon-beta-associated-thrombotic-microangiopathy-following-treatment-with-corticosteroids-plasma-exchange-and-rituximab-a-case-report
#1
Lea M Gerischer, Eberhard Siebert, Oliver Janke, Gerhard Jan Jungehuelsing, Klemens Ruprecht
Thrombotic microangiopathy (TMA) is a rare but increasingly recognized complication of interferon-beta therapy, which can be associated with serious sequelae. We report on a 53-year-old woman with a longstanding history of relapsing-remitting multiple sclerosis, who developed TMA after 15 years of high-dose treatment with subcutaneous interferon-beta-1a. The patient presented with headaches, an epileptic seizure, confusion, and arterial hypertension. Laboratory findings included thrombocytopenia and hemolytic anemia...
November 2016: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/27918307/vegf-regulates-local-inhibitory-complement-proteins-in-the-eye-and-kidney
#2
Lindsay S Keir, Rachel Firth, Lyndsey Aponik, Daniel Feitelberg, Susumu Sakimoto, Edith Aguilar, Gavin I Welsh, Anna Richards, Yoshihiko Usui, Simon C Satchell, Valeryia Kuzmuk, Richard J Coward, Jonathan Goult, Katherine R Bull, Ruchi Sharma, Kapil Bharti, Peter D Westenskow, Iacovos P Michael, Moin A Saleem, Martin Friedlander
Outer retinal and renal glomerular functions rely on specialized vasculature maintained by VEGF that is produced by neighboring epithelial cells, the retinal pigment epithelium (RPE) and podocytes, respectively. Dysregulation of RPE- and podocyte-derived VEGF is associated with neovascularization in wet age-related macular degeneration (ARMD), choriocapillaris degeneration, and glomerular thrombotic microangiopathy (TMA). Since complement activation and genetic variants in inhibitory complement factor H (CFH) are also features of both ARMD and TMA, we hypothesized that VEGF and CFH interact...
December 5, 2016: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27913748/a-3-year-follow-up-of-a-patient-with-acute-renal-failure-caused-by-thrombotic-microangiopathy-related-to-antiphospholipid-syndrome-case-report
#3
X-J Zhou, M Chen, S-X Wang, F-D Zhou, M-H Zhao
BACKGROUND: Microvascular manifestations of antiphospholipid antibody syndrome in the kidneys include acute renal failure, thrombotic microangiopathy and hypertension. Therapy has been largely empiric. CASE REPORT: A 49-year-old Chinese man presented with anuric acute renal failure without abundant proteinuria and heavy haematuria, but markedly low levels of urinary sodium, potassium and chlorine upon admission. On day 1 of hospitalization, his thrombocytopenia, anaemia and renal failure showed rapid progression...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27913483/atypical-hemolytic-uremic-syndrome
#4
Vahid Afshar-Kharghan
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) that affects multiple organs and the kidneys in particular. aHUS can be sporadic or familial and is most commonly caused by dysregulation of the alternative complement pathway. The initial attack of aHUS can occur at any age, and is associated with a high rate of progression to end stage renal disease. Many aHUS patients relapse in the native or transplanted kidneys, and require close monitoring and long-term management. Availability of anticomplement therapy has revolutionized the management of aHUS, and can change the natural course of aHUS by inducing hematologic remission, improving or stabilizing kidney functions, and preventing graft failure...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#5
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27892947/danaparoid-reduces-the-incidence-of-hematopoietic-stem-cell-transplantation-associated-thrombotic-microangiopathy
#6
S Machida, M Onizuka, M Toyosaki, Y Aoyama, H Kawai, J Amaki, R Hara, A Ichiki, Y Ogawa, H Kawada, K Ando
No abstract text is available yet for this article.
November 28, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27887777/adamts13-specific-circulating-immune-complexes-as-potential-predictors-of-relapse-in-patients-with-acquired-thrombotic-thrombocytopenic-purpura
#7
Ilaria Mancini, Barbara Ferrari, Carla Valsecchi, Silvia Pontiggia, Marco Fornili, Elia Biganzoli, Flora Peyvandi
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy due to the development of autoantibodies against the VWF-cleaving protease ADAMTS13. ADAMTS13-specific circulating immune complexes (CICs) have been described in patients with acquired TTP, but their clinical relevance remained to be established. The aim of this study was to assess the association between ADAMTS13-specific CICs and ADAMTS13-related measurements, clinical and laboratory markers of disease severity, and occurrence of TTP relapse, in autoimmune TTP patients...
November 22, 2016: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/27885340/acute-kidney-injury-in-hematopoietic-stem-cell-transplantation-a-review
#8
REVIEW
Vinod Krishnappa, Mohit Gupta, Gurusidda Manu, Shivani Kwatra, Osei-Tutu Owusu, Rupesh Raina
Hematopoietic stem cell transplantation (HSCT) is a highly effective treatment strategy for lymphoproliferative disorders and bone marrow failure states including aplastic anemia and thalassemia. However, its use has been limited by the increased treatment related complications, including acute kidney injury (AKI) with an incidence ranging from 20% to 73%. AKI after HSCT has been associated with an increased risk of mortality. The incidence of AKI reported in recipients of myeloablative allogeneic transplant is considerably higher in comparison to other subclasses mainly due to use of cyclosporine and development of graft-versus-host disease (GVHD) in allogeneic groups...
2016: International Journal of Nephrology
https://www.readbyqxmd.com/read/27884308/talking-back-the-podocytes-and-endothelial-cells-duke-it%C3%A2-out
#9
Agnes B Fogo
Thrombotic microangiopathy has numerous causes and may result in chronic kidney disease with secondary glomerulosclerosis. Detailed analyses of this interplay of lesions have been lacking. Buob et al. report on their adult, mostly Caucasian patients, showing frequent sclerosis, most often of collapsing type, with worse prognosis than in those without segmental scars. The complex interplay of glomerular cells and possible ways in which the endothelial cells may talk back to the podocytes, and vice versa, are discussed...
December 2016: Kidney International
https://www.readbyqxmd.com/read/27884283/ajkd-atlas-of-renal-pathology-thrombotic-microangiopathy
#10
Mark A Lusco, Agnes B Fogo, Behzad Najafian, Charles E Alpers
No abstract text is available yet for this article.
December 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27882637/transplantation-associated-thrombotic-microangiopathy-isolated-to-a-congenital-anomaly-of-the-lung
#11
Rebecca T Kummen, Geoffrey D E Cuvelier, Camelia Stefanovici, Anamarija M Perry, Rick Higgins, Rochelle Yanofsky, Suyin A Lum Min, Donna A Wall
TA-TMA is a post-hematopoietic stem cell transplant complication with clinical features of hemolytic anemia and thrombocytopenia. A 26-month-old child who had had an allogeneic transplant for treatment of DBA developed severe TA-TMA with heavy red blood cell and platelet transfusion dependence. Incidentally, he was found to have a lung sequestration. TA-TMA resolved and transfusion dependence resolved after resection of the sequestration. The finding suggests the malformation vasculature was selectively vulnerable to the trigger of TA-TMA-raising perhaps a clue to basic pathophysiology of TA-TMA and/or vascular malformations...
November 24, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27878664/diagnosis-and-management-of-acquired-thrombotic-thrombocytopenic-purpura-in-southeast-china-a-single-center-experience-of-60-cases
#12
Xinping Zhou, Xingnong Ye, Yanling Ren, Chen Mei, Liya Ma, Jiansong Huang, Weilai Xu, Juying Wei, Li Ye, Wenyuan Mai, Wenbin Qian, Haitao Meng, Jie Jin, Hongyan Tong
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy. This study aimed to provide a profile of the diagnosis and management of patients with acquired TTP collected in 10 years in a single center in southeast China. A total of 60 patients diagnosed with acute acquired TTP from March 2005 to August 2015 were enrolled. Among the 60 patients, 52 patients presented with their first episodes, and eight patients had two or more episodes. The median age at presentation was 49 (range, 17 to 78) years with a female predominance (male:female ratio, 1:1...
November 23, 2016: Frontiers of Medicine
https://www.readbyqxmd.com/read/27868334/consensus-on-the-standardization-of-terminology-in-thrombotic-thrombocytopenic-purpura-and-related-thrombotic-microangiopathies
#13
M Scully, S Cataland, P Coppo, J de la Rubia, K D Friedman, J Kremer Hovinga, B Lämmle, M Matsumoto, K Pavenski, E Sadler, R Sarode, H Wu
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic Microangiopathy is a broad pathophysiological process that leads to microangiopathic hemolytic anemia, thrombocytopenia and involves capillary and small vessel platelet aggregates. The most common cause being disseminated intravascular coagulation (DIC), which may be differentiated by abnormal coagulation. Clinically, a number of conditions present with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT), including cancer, infection, transplantation, drugs, autoimmune disease and pre-eclampsia and HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome in pregnancy...
November 21, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27865549/anaemia-and-fever-in-kidney-transplant-the-role-of-human-parvovirus-b19
#14
Yanet Parodis López, Raquel Santana Estupiñán, Silvia Marrero Robayna, Roberto Gallego Samper, Fernando Henríquez Palop, José Carlos Rivero Vera, Rafael Camacho Galán, María José Pena López, Nery Sablón González, Fayna González Cabrera, Elena Oliva Dámaso, Nicanor Vega Díaz, José Carlos Rodríguez Pérez
Infections remain an issue of particular relevance in renal transplant patients, particularly viral infections. Human parvovirus B19 infection causes severe refractory anaemia, pancytopenia and thrombotic microangiopathy. Its presence is recognized by analysing blood polymerase chain reaction (PCR) and by the discovery of typical giant proerythroblasts in the bone marrow. We report the case of a 65 year-old man with a history of deceased donor renal transplant in September 2014. At 38 days after the transplant, the patient presented progressive anaemia that was resistant to erythropoiesis-stimulating agents...
November 16, 2016: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/27864296/a-mechanistic-investigation-of-thrombotic-microangiopathy-associated-with-intravenous-abuse-of-opana-er
#15
Ryan Hunt, Ayla Yalamanoglu, James Tumlin, Tal Schiller, Jin Hyen Baek, Andrew Wu, Agnes B Fogo, Haichun Yang, Edward Wong, Peter Miller, Paul W Buehler, Chava Kimchi-Sarfaty
Since 2012, a number of case reports have described the occurrence of thrombotic microangiopathy (TMA) following intravenous abuse of extended release oxymorphone hydrochloride (Opana ER), an oral opioid for long-term treatment of chronic pain. Here, we present unique clinical features of three patients and investigate intravenous exposure to the tablet's inert ingredients as a possible causal mechanism. Guinea pigs were used as an animal model to understand the hematopathologic and nephrotoxic potential of the inert ingredient mixture (termed here as PEO+) which primarily contains high molecular weight polyethylene oxide (HMW PEO)...
November 18, 2016: Blood
https://www.readbyqxmd.com/read/27861383/living-donor-renal-transplantation-in-patients-with-antiphospholipid-syndrome-a-case-report
#16
Ji Yoon Choi, Joo Hee Jung, Sung Shin, Young Hoon Kim, Duck Jong Han
INTRODUCTION: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. CLINICAL FINDINGS: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27848226/atypical-hemolytic-uremic-syndrome-in-the-setting-of-complement-amplifying-conditions-case-reports-and-a-review-of-the-evidence-for-treatment-with-eculizumab
#17
REVIEW
Arif Asif, Ali Nayer, Christian S Haas
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, progressive, life-threatening form of thrombotic microangiopathy (TMA) predominantly caused by dysregulation of the alternative pathway of the complement system. Complement-amplifying conditions (CACs), including pregnancy complications [preeclampsia, HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome], malignant hypertension, autoimmune diseases, transplantation, and others, are associated with the onset of TMA in up to 69 % of cases of aHUS...
November 15, 2016: Journal of Nephrology
https://www.readbyqxmd.com/read/27830476/production-of-heterozygous-alpha-1-3-galactosyltransferase-ggta1-knock-out-transgenic-miniature-pigs-expressing-human-cd39
#18
Kimyung Choi, Joohyun Shim, Nayoung Ko, Heejong Eom, Jiho Kim, Jeong-Woong Lee, Dong-Il Jin, Hyunil Kim
Production of transgenic pigs for use as xenotransplant donors is a solution to the severe shortage of human organs for transplantation. The first barrier to successful xenotransplantation is hyperacute rejection, a rapid, massive humoral immune response directed against the pig carbohydrate GGTA1 epitope. Platelet activation, adherence, and clumping, all major features of thrombotic microangiopathy, are inevitable results of immune-mediated transplant rejection. Human CD39 rapidly hydrolyzes ATP and ADP to AMP; AMP is hydrolyzed by ecto-5'-nucleotidase (CD73) to adenosine, an anti-thrombotic and cardiovascular protective mediator...
November 9, 2016: Transgenic Research
https://www.readbyqxmd.com/read/27824770/microangiopathic-hemolytic-anemia-due-to-malignancy-in-pregnancy
#19
Sarah K Happe, Amanda C Zofkie, David B Nelson
BACKGROUND: Microangiopathic disorders during pregnancy remain a diagnostic challenge because these conditions often imitate more common obstetric diseases. CASE: A 31-year-old multigravid woman delivered at term with a postpartum course complicated by laboratory indices consistent with hemolysis, elevated liver enzymes, and low platelet count syndrome. After clinical recovery, she was readmitted 2 weeks after delivery with presumptive pyelonephritis. Persistent hemolysis and worsening thrombocytopenia suggested a diagnosis of thrombotic microangiopathy, which did not respond to plasma exchange...
December 2016: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27823643/an-overview-of-hematopoietic-stem-cell-transplantation-related-thrombotic-complications
#20
REVIEW
Ugur Sahin, Pinar Ataca Atilla, Erden Atilla, Selami Kocak Toprak, Taner Demirer
Thrombotic episodes are far less common than bleeding complications after hematopoietic stem cell transplantation (HSCT). However, they lead to significant morbidity and mortality. These complications are classified into four groups, including venous thromboembolic events (VTE), catheter-induced thrombosis (CIT), transplant-associated thrombotic microangiopathy (TA-TMA) and sinusoidal obstruction syndrome (SOS) or veno-occlusive disease (VOD). The frequency of VTE is increased among patients undergoing HSCT due to some acquired conditions including underlying malignancy, infections, administration of myeloablative conditioning regimens and/or total body irradiation, prolonged hospitalizations leading to immobility and presence of central venous catheters...
November 2016: Critical Reviews in Oncology/hematology
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