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thrombotic microangiopathies

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https://www.readbyqxmd.com/read/28626544/atypical-hemolytic-uremic-syndrome-a-brief-review
#1
REVIEW
Kuixing Zhang, Yuxin Lu, Kevin T Harley, Minh-Ha Tran
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28621343/current-evidence-on-the-discontinuation-of-eculizumab-in-patients-with-atypical-haemolytic-uraemic-syndrome
#2
COMMENT
Manuel Macia, Fernando de Alvaro Moreno, Tina Dutt, Ingela Fehrman, Karine Hadaya, Christoph Gasteyger, Nils Heyne
Background. Atypical haemolytic uraemic syndrome (aHUS) is a rare, life-threatening disorder for which eculizumab is the only approved treatment. Life-long treatment is indicated; however, eculizumab discontinuation has been reported. Methods. Unpublished authors' cases and published cases of eculizumab discontinuation are reviewed. We also report eculizumab discontinuation data from five clinical trials, plus long-term extensions and the global aHUS Registry. Results. Of six unpublished authors' cases, four patients had a subsequent thrombotic microangiopathy (TMA) manifestation within 12 months of discontinuation...
June 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28614243/different-types-of-glomerulonephritis-associated-with-the-dysregulation-of-the-complement-alternative-pathway-in-2-brothers-a-case-report
#3
Pei Chen, Li Zhu, Feng Yu, Sha-Sha Han, Si-Jun Meng, Wei-Yi Guo, Hong Zhang, Yan Song
RATIONALE: C3 glomerulonephritis (C3GN) and complement-mediated hemolytic uremic syndrome (HUS) both result from the abnormal regulation of the complement system. A significant number of patients with C3GN or complement-mediated HUS have mutations of more than 1 complement protein. This discovery has had a major impact on identifying the underlying cause of familial C3GN or complement-mediated HUS. PATIENT CONCERNS: We report the cases of 2 brothers (herein referred to as patient II-1 and patient II-9), both with complement disorders that differed in their clinical and genetic features...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28612003/discontinuation-of-peritoneal-dialysis-after-late-initiation-of-eculizumab-in-a-case-of-familial-atypical-hemolytic-uremic-syndrome-a-case-report
#4
Rafael Alonso Valente, Giannina Elena García Rodríguez, Yanina García Marcote, Manuel Fidalgo Díaz, Vanesa Becerra Mosquera, Daniel Novoa García, Teresa Cordal Martínez, Cándido Díaz Rodríguez
BACKGROUND: Atypical hemolytic-uremic syndrome is caused by a thrombotic microangiopathy and manifests itself with hemolytic anemia, thrombocytopenia, and organ ischemia. Its etiology is a mutation affecting the genes encoding for proteins of the complement system. Early treatment with eculizumab (8.6 months from the moment of presentation), a humanized monoclonal antibody against complement, is shown to be effective in controlling symptoms and reversing organ damage. We present a patient with a mutation not previously described in the literature...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28611987/complement-in-kidney-transplantation
#5
REVIEW
Marek Cernoch, Ondrej Viklicky
The complement system is considered to be an important part of innate immune system with a significant role in inflammation processes. The activation can occur through classical, alternative, or lectin pathway, resulting in the creation of anaphylatoxins C3a and C5a, possessing a vast spectrum of immune functions, and the assembly of terminal complement cascade, capable of direct cell lysis. The activation processes are tightly regulated; inappropriate activation of the complement cascade plays a significant role in many renal diseases including organ transplantation...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28607917/thrombotic-microangiopathy-associated-with-synthetic-cannabinoid-receptor-agonists
#6
Michael Karass, Savneek Chugh, Gabriela Andries, Aleksandra Mamorska-Dyga, John C Nelson, Praveen N Chander
Marijuana is one of the most commonly used recreational drugs in the United States. As marijuana is illegal in the majority of countries, the use of readily available and unregulated synthetic cannabinoids (SCBs) has increased. Little is known about the potential adverse effects of SCBs especially in regards to their nephrotoxicity. Case reports of acute kidney injury (AKI) from acute tubular injury secondary to their use have been reported. However, the exact pathology, mechanism, and extent of renal injury remain unknown...
2017: Stem Cell Investigation
https://www.readbyqxmd.com/read/28607779/thrombotic-microangiopathy-secondary-to-intravenous-abuse-of-opana%C3%A2-er
#7
Kamia Thakur, Vaibhav Agrawal, Ashley Kass, Lauren M Dimarino, R Patrick Dorion, Joseph Vadakara
Opana ER (oxymorphone) is an opioid drug available throughout the United States, and intravenous abuse of the crushed oral formulation has been associated with drug-induced thrombotic microangiopathy. In this abstract, we describe two young patients who lived together and used Opana ER intravenously. Both presented with microangiopathic hemolytic anemia that mimicked thrombotic thrombocytopenic purpura (TTP). Treating this condition poses a clinical challenge, as it is difficult to distinguish it from TTP. The role for plasma exchange is not clear but can be used while awaiting the results of the ADAMTS-13 activity, but ultimately supportive care with drug discontinuation is the recommended therapy of choice...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28597458/clinical-scoring-systems-in-thrombotic-microangiopathies
#8
Pavan K Bendapudi, Vivek Upadhyay, Lova Sun, Marisa B Marques, Robert S Makar
No abstract text is available yet for this article.
July 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28596415/hemolytic-uremic-syndrome-in-pregnancy-and-post-partum
#9
Alexandra Bruel, David Kavanagh, Marina Noris, Yahsou Delmas, Edwin K S Wong, Elena Bresin, François Provôt, Vicky Brocklebank, Caterina Mele, Giuseppe Remuzzi, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Pregnancy is associated with various forms of thrombotic microangiopathy, including hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical hemolytic uremic syndrome linked to complement alternative pathway dysregulation. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway gene variants in a larger international (France, United Kingdom, Italy) cohort of patients with pregnancy-associated hemolytic uremic syndrome...
June 8, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28596255/thrombotic-thrombocytopenic-purpura-associated-with-pegylated-interferon-alfa-2a-use-in-a-patient-with-polycythemia-vera
#10
Radhika Gangaraju, Soo J Kim, Jing-Fei Dong, Sabina Swierczek, Josef T Prchal
Pegylated interferon alfa-2a (pegIFNa) is being increasingly used for treatment of myeloproliferative neoplasms; however, its side effects, including autoimmune complications, are not unusual. We report on a 47-year-old woman with polycythemia vera (PV) treated with pegIFNa and in complete hematologic remission who developed thrombotic thrombocytopenic purpura (TTP). To our knowledge, thrombotic microangiopathy has been reported as a side effect of interferon (IFN) use in patients with hepatitis and chronic myeloid leukemia, but not in those with PV...
June 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28596204/malignant-hypertension-as-a-rare-cause-of-thrombotic-microangiopathy
#11
Guramrinder Thind, Karthik Kailasam
Malignant hypertension can occasionally be associated with microangiopathic haemolytic anaemia. A 38-year-old male presented with nausea, vomiting, loss of appetite and oliguria for 2 weeks. He was diagnosed with hypertensive emergency with cardiac and renal dysfunction. Interestingly, further workup was diagnostic for the presence of thrombotic microangiopathy (TMA): haemoglobin =12.7 g/dL, indirect bilirubin =2.0 mg/dL, haptoglobin ≤6 mg/dL, platelet count =121 000/μL and schistocytes on peripheral smear...
June 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28593773/-hellp-syndrome-requiring-therapeutic-plasma-exchange-due-to-progression-to-multiple-organ-dysfunction-syndrome-with-predominant-encephalopathy-respiratory-and-renal-insufficiency
#12
M Trávniková, J Gumulec, Z Kořístek, M Navrátil, M Janáč, J Pelková, P Šuráň, E Doležálková, O Šimetka
OBJECTIVE: Case report of woman with twin pregnancy complicated by HELLP syndrome which progressed to multiple organ dysfunction syndrome with predominant encephalopathy, renal and respiratory insufficiency with the need to perform repeated therapeutic plasma exchange. DESIGN: Case report. SETTING: Department of gynecology and obstetrics, University Hospital in Ostrava; Departmet of hematooncology, University Hospital in Ostrava; Department of gynecology and obstetrics, Vsetín hospital; Department of hematology and transfusion, Vsetín Hospital...
2017: Ceská Gynekologie
https://www.readbyqxmd.com/read/28592771/ttp-and-ahus-new-insights
#13
Yoshitaka Miyakawa
Both TTP and aHUS are officially designated as intractable diseases by a new Japanese law in 2015. New clinical practice guidelines for TTP and aHUS have been published. Both conditions share some common pathophysiology such as thrombotic microangiopathy. Unfortunately, most professionals in the fields of hematology/oncology are unaware of these diseases. Therefore, I will provide an updated overview of TTP and aHUS in this article.
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28592769/thrombotic-complications-after-hematopoietic-stem-cell-transplantation
#14
Shosaku Nomura
Hematopoietic stem cell transplantation (HSCT) involves some serious transplant-associated complications (TACs) or vascular disorders, such as veno-occlusive disease (VOD), thrombotic microangiopathy (TMA), and graft-versus-host disease (GVHD). VOD is related to a clinical syndrome characterized by tender hepatomegaly, jaundice, fluid retention, and unexplained weight gain. When TMA is described in patients who have undergone HSCT, it is often implied that the clinical diagnosis of TMA is similar to that of thrombotic thrombocytopenic purpura...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28580158/opana-induced-thrombotic-microangiopathy-masquerading-as-thrombotic-thrombocytopenic-purpura
#15
Byung H Ban, Ashish Verma, Maria Tudor, Jigme Sethi
Opana (oxymorphone) is a powerful semi-synthetic opioid agonist used for chronic pain management that is ingested orally. However, improper injection of Opana can lead to a rare and fatal blood disorder known as thrombotic microangiopathy. Opana-induced thrombotic microangiopathy can be easily mistaken for thrombotic thrombocytopenic purpura (TTP), leading to the initiation of therapeutic plasma exchange. Current literature has conflicting views on the necessity of therapeutic plasma exchange for the treatment of Opana-induced thrombotic microangiopathy...
June 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28578979/hiv-associated-kidney-diseases-clarifying-concordance-between-renal-failure-in-hiv-infection-and-histopathologic-manifestations-at-kidney-biopsy
#16
REVIEW
Carla L Ellis
Patients with HIV infection have a wide spectrum of renal diseases. Some are known to be the direct effect of the viral infection while others are renal diseases that also occur in uninfected populations. HIV associated nephropathy (HIVAN) is considered to be a subtype of primary focal and segmental glomerulosclerosis that is distinct in HIV infected patients. It is more frequent in the African-American population and associated with mutations of the apolipoprotein L1 (APOL1) gene. HIV associated immune complex kidney disease (HIVICD) encompasses a spectrum of HIV associated renal diseases characterized by the presence of immune complex deposition within glomeruli...
May 5, 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28577731/thrombotic-microangiopathies-and-antineoplastic-agents
#17
Steven Grangé, Paul Coppo
Thrombotic microangiopathy is a well-described complication of cancer treatment. Its incidence has increased these last decades, as a result of a better awareness of this complication in cancer patients in one hand, but also of a larger array of therapeutic compounds including anti-vascular endothelium growth factor (VEGF) drugs. It is therefore mandatory to recognize these conditions since they have a significant impact in thrombotic microangiopathies management and prognosis. Practitioners should be aware of the more classical antineoplastic agents associated with thrombotic microangiopathies, the mechanisms by which they induce them, and the resulting management and prognosis...
April 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28575423/purtscher-like-retinopathy-and-transplant-associated-thrombotic-microangiopathy
#18
Satoko Tominaga, Shinji Makino
No abstract text is available yet for this article.
May 29, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28542627/predictive-features-of-chronic-kidney-disease-in-atypical-haemolytic-uremic-syndrome
#19
Matthieu Jamme, Quentin Raimbourg, Dominique Chauveau, Amélie Seguin, Claire Presne, Pierre Perez, Pierre Gobert, Alain Wynckel, François Provôt, Yahsou Delmas, Christiane Mousson, Aude Servais, Laurence Vrigneaud, Agnès Veyradier, Eric Rondeau, Paul Coppo
Chronic kidney disease (CKD) is a frequent and serious complication of atypical haemolytic uremic syndrome (aHUS). We aimed to develop a simple accurate model to predict the risk of renal dysfunction in aHUS based on clinical and biological features available at hospital admission. Renal function at 1-year follow-up, based on an estimated glomerular filtration rate < 60mL/min/1.73m2 as assessed by the Modification of Diet in Renal Disease equation, was used as an indicator of significant CKD. Prospectively collected data from a cohort of 156 aHUS patients who did not receive eculizumab were used to identify predictors of CKD...
2017: PloS One
https://www.readbyqxmd.com/read/28529915/hemolytic-uremic-syndrome-in-adults-a-case-report
#20
Fabiel Gerardo Pérez-Cruz, Patricia Villa-Díaz, María Consuelo Pintado-Delgado, María Loreto Fernández Rodríguez, Ana Blasco-Martínez, María Pérez-Fernández
Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by platelet aggregation and mechanical damage to erythrocytes, clinically characterized by microangiopatic haemolytic anemia, thrombocytopenia and organ injury. We are reporting a case of a woman patient with severe hemolytic uremic syndrome associated to infectious diarrhoea caused by Shiga toxin-producing pathogen, who were admitted to our intensive care unit. The patient described developed as organ injury, neurological failure and acute renal failure, with need of haemodialysis technique...
May 4, 2017: World Journal of Critical Care Medicine
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