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ADAMTS13

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https://www.readbyqxmd.com/read/28443948/relapse-of-congenital-thrombotic-thrombocytopenic-purpura-after-spontaneous-remission-in-a-second-trimester-primigravida-case-report-and-review-of-the-literature
#1
Donavan de Souza Lúcio, Jacqueline Foelkel Pignatari, Marcelo Gil Cliquet, Henri Augusto Korkes
CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities...
April 20, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28428179/adamts13-controls-vascular-remodeling-by-modifying-vwf-reactivity-during-stroke-recovery
#2
Haochen Xu, Yongliang Cao, Xing Yang, Ping Cai, Lijing Kang, Ximin Zhu, Haiyu Luo, Lu Lu, Lixiang Wei, Xiaofei Bai, Yuanbo Zhu, Bing-Qiao Zhao, Wenying Fan
Angiogenic response is essential for ischemic brain repair. However, the mechanisms leading to postischemic neovascularization have not been established, and no therapies have been identified. The VWF-cleaving protease ADAMTS13 is required for endothelial tube formation in vitro However, there is currently no in vivo evidence supporting a function of ADAMTS13 in angiogenesis. Here we show that mice deficient in ADAMTS13 exhibited significantly reduced neovascularization, brain capillary perfusion, pericyte and smooth muscle cell coverage on microvessels, expression of the tight junction and basement membrane proteins, and accelerated blood-brain barrier (BBB) breakdown and extravascular deposits of serum proteins in the peri-infarct cortex at 14 days after stroke...
April 20, 2017: Blood
https://www.readbyqxmd.com/read/28416509/none-of-the-above-thrombotic-microangiopathy-beyond-ttp-and-hus
#3
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#4
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28383422/acquired-idiopathic-thrombotic-thrombocytopenic-purpura-successfully-treated-with-intravenous-immunoglobulin-and-glucocorticoid-a-case-report
#5
Hiro Nakao, Akira Ishiguro, Nahoko Ikoma, Kentaro Nishi, Chemin Su, Hisaya Nakadate, Mitsuru Kubota, Masaki Hayakawa, Masanori Matsumoto
RATIONALE: Plasma exchange is the principal treatment for acquired thrombotic thrombocytopenic purpura (TTP) but is invasive and may have adverse effects. Reports of immunoglobulin therapy for acquired TTP without plasma exchange are rare. PATIENT CONCERNS: A 14-year-old girl was admitted because of hemolytic anemia and thrombocytopenia. DIAGNOSIS: Acquired TTP was diagnosed based on low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13) activity and a high ADAMTS13 inhibitor level...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#6
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28382235/influences-of-abo-blood-group-age-and-gender-on-plasma-coagulation-factor-viii-fibrinogen-von-willebrand-factor-and-adamts13-levels-in-a-chinese-population
#7
Zongkui Wang, Miaomiao Dou, Xi Du, Li Ma, Pan Sun, Haijun Cao, Shengliang Ye, Peng Jiang, Fengjuan Liu, Fangzhao Lin, Rong Zhang, Changqing Li
BACKGROUND: ABO blood group is a hereditary factor of plasma levels of coagulation factor VIII (FVIII) and von Willebrand factor (VWF). Age and gender have been shown to influence FVIII, VWF, fibrinogen (Fbg), and ADAMTS13 (A disintegrin and metalloprotease with thrombospondin type 1 motif, 13). We investigated the effects of ABO type, age, and gender on plasma levels of FVIII, Fbg, VWF, and ADAMTS13 in a Chinese population. METHODS: A total of 290 healthy volunteers were eligible for this study...
2017: PeerJ
https://www.readbyqxmd.com/read/28381686/refractory-thrombotic-thrombocytopenic-purpura-achieving-complete-remission-with-rituximab-treatment
#8
Hiromasa Iino, Yoshiyuki Ogawa, Kunio Yanagisawa, Hiroaki Shimizu, Takeki Mitsui, Takuma Ishizaki, Masaki Hayakawa, Masanori Matsumoto, Yoshihisa Nojima, Hiroshi Handa
Some patients with thrombotic thrombocytopenic purpura (TTP) are refractory to standard treatment regimens comprised of plasma exchange (PEX) and steroids. This report describes a 40-year-old woman with refractory TTP who achieved complete remission (CR) in response to rituximab. She was referred to our institution from a rural hospital with purpura of the extremities, severe thrombocytopenia, anemia, and rapidly progressive disturbance of consciousness. TTP was diagnosed based on the clinical symptoms of TTP, low ADAMTS13 activity (<0...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28370891/adamts13-glycans-and-conformation-dependent-activity
#9
A A Nowak, H E R O'Brien, P Henne, A Doerr, K Vanhoorelbeke, M A Laffan, T A J McKinnon
BACKGROUND: ADAMTS13 activity can be regulated by its conformation where interactions between the C-terminal CUB domains and the Spacer domain maintain ADAMTS13 in a closed conformation. ADAMTS13 contains 10 N-linked glycans, with four sites present in the TSP2 through CUB domains that may contribute to its conformation. OBJECTIVES/METHODS: We hypothesized that glycosylation contributes to ADAMTS13 conformation and function. The proteolytic activity of glycan modified ADAMTS13 was assessed under static and shear stress conditions...
March 31, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28369949/evaluating-the-impact-of-the-abo-blood-group-on-the-clinical-outcome-of-thrombotic-thrombocytopenic-purpura-associated-with-severe-adamts13-deficiency
#10
E Hussein, J Teruya
BACKGROUND AND OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) is caused by the decrease of ADAMTS13, leading to the accumulation of ultra large von Willebrand factor (ULVWF). It was proposed that the distribution of blood group O among TTP patients may be potentially lower than expected because of the lower levels of VWF. The aim of this study was to explore the relationship between various blood groups and the clinical outcome in TTP. MATERIALS AND METHODS: Thirty-three patients with TTP with severe ADAMTS13 deficiency were studied...
March 31, 2017: Vox Sanguinis
https://www.readbyqxmd.com/read/28367342/remission-of-severe-relapsed-and-refractory-ttp-after-multiple-cycles-of-bortezomib
#11
Manu R Pandey, Pankit Vachhani, Evelena P Ontiveros
Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by autoantibodies against a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Uncleaved von Willebrand factor (VWF) multimers accumulate and bind to platelets which causes spontaneous microthrombi ultimately causing microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemia. Plasma exchange (PEX) with or without steroids constitutes standard first-line therapy with rituximab typically reserved for refractory cases...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28355731/-the-455th-case-swollen-leg-jaundice-and-mental-disturbance
#12
R Dong, L Weng, T Guo, T N Zhu, J L Zhao, Q J Wu, X F Zeng
A 17-year-old young man with a history of swollen leg and intermittent jaundice was presented to Peking Union Medical College Hospital with acute fever and mental disturbance. He developed deep venous thrombosis, acute myocardial infarction and plantar skin necrosis during the past four years, and was presented with an acute episode of fever, thrombocytopenia, acute kidney injury, acute myocardial infarction, mental disturbance, and obstructive jaundice. Laboratory tests showed schistocytes on peripheral blood smear...
April 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28352025/eculizumab-experience-in-an-adult-patient-with-atypical-hemolytic-uremic-syndrome
#13
Funda Sari, Ayça Inci, Volkan Karakus, Banu Yilmaz, Metin Sarikaya, Refik Olmaz, Erdal Kurtoglu
Atypical hemolytic-uremic syndrome is a disease characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal failure. In this study, we present a case of a patient with atypical hemolytic-uremic syndrome treated successfully with eculizumab. A 20-year-old female was admitted with clinical signs of atypical hemolytic syndrome. The laboratory findings were as follows: hemoglobin 9.2 g/dL, platelet count 18 × 103/μL, creatinine 4.69 mg/dL, schistocytes were in peripheral blood smear, lactate dehydrogenase 2080 U/L, and emergency plasmapheresis procedure with fresh frozen plasma were initiated...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28339879/high-frequency-of-mutations-in-genes-encoding-factor-viii-von-willebrand-factor-and-adamts13-in-skin-cutaneous-melanoma
#14
Zheng Ping, Huy P Pham, Lance A Williams, X Long Zhen
No abstract text is available yet for this article.
March 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28339859/human-neutrophil-peptides-hnps-inhibit-proteolytic-cleavage-of-von-willebrand-factor-by-adamts13-a-potential-link-between-inflammation-and-thrombotic-thrombocytopenic-purpura
#15
Vikram G Pillai, Jialing Bao, Catherine B Zander, Jenny McDaniel, Steven H Seeholzer, Khalil Bdeir, Douglas B Cines, X Long Zhen
No abstract text is available yet for this article.
March 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28326725/-analysis-of-salivary-protease-spectrum-in-chronic-periodontitis
#16
Li Qian, Zhou Xuedong, Fan Yaping, Yang Tengyu, Wu Songtao, Yu Yu, Chen Jiao, Zhang Ping, Feng Yun
OBJECTIVE: This study aimed to investigate the difference in salivary protease expression in patients with chronic periodontitis and normal individuals. METHODS: The stimulating saliva in patients with chronic periodontitis and normal individuals were collected. Protein chip technology was adapted to analyze salivary protease spectrum. RESULTS: Among the 34 proteases in the chip, disintegrin and metalloproteinase (ADAM)8, matrix metalloproteinase (MMP)-8, MMP-12, neprilysin/CD10, and uridylyl phosphate adenosine/urokinase showed a significantly increased concentration in the saliva of chronic periodontitis patients compared with those in the saliva of normal individuals (P<0...
February 1, 2017: Hua Xi Kou Qiang Yi Xue za Zhi, Huaxi Kouqiang Yixue Zazhi, West China Journal of Stomatology
https://www.readbyqxmd.com/read/28321086/acquired-thrombotic-thrombocytopenic-purpura-with-a-smoldering-clinical-course
#17
Chiaki Naito, Yoshiyuki Ogawa, Kunio Yanagisawa, Takuma Ishizaki, Masahiro Mihara, Masaki Hayakawa, Masanori Matsumoto, Yoshihisa Nojima, Hiroshi Handa
Acquired thrombotic thrombocytopenic purpura (aTTP) is caused by a deficiency of ADAMTS13 activity due to neutralizing auto-autoantibodies (inhibitors) against ADAMTS13. Patients with aTTP show a rapid and fatal clinical course, unless an effective therapeutic intervention such as plasma exchange therapy (PEX) is performed. There is, however, a small population of patients who show a smoldering clinical course, for which no effective treatment strategy has yet been established. We herein report a 77-year-old man, who had repeated episodes of cerebral infarction and persistent thrombocytopenia over several months, but was not correctly diagnosed as having aTTP at a local hospital...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28296884/increased-von-willebrand-factor-decreased-adamts13-and-thrombocytopenia-in-melioidosis
#18
Emma Birnie, Gavin C K W Koh, Ester C Löwenberg, Joost C M Meijers, Rapeephan R Maude, Nicholas P J Day, Sharon J Peacock, Tom van der Poll, W Joost Wiersinga
BACKGROUND: Melioidosis, caused by bioterror treat agent Burkholderia pseudomallei, is an important cause of community-acquired Gram-negative sepsis in Southeast Asia and Northern Australia. New insights into the pathogenesis of melioidosis may help improve treatment and decrease mortality rates from this dreadful disease. We hypothesized that changes in Von Willebrand factor (VWF) function should occur in melioidosis, based on the presence of endothelial stimulation by endotoxin, pro-inflammatory cytokines and thrombin in melioidosis, and investigated whether this impacted on outcome...
March 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28283300/microangiopathic-hemolytic-anemia-in-pregnancy
#19
Mohammed Salhab, Andrew Hsu, Elizabeth Ryer, Juliet Appiah, Bradley Switzer
Microangipathic hemolytic anemia (MAHA) is a serious diagnosis and difficult to manage in pregnant patients as multiple life threatening conditions could present with MAHA. ADAMTS13 enzyme activity can be affected during pregnancy with multiple factors. A persistent extremely low ADAMTS13 enzyme activity levels, without an inhibitor, after the delivery was an important factor to establish the diagnosis. We present a case of likely congenital ADAMST13 deficiency that manifested for the first time in a pregnant woman at week 37 of pregnancy...
February 16, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28279966/plasmin-cleaves-von-willebrand-factor-at-k1491-r1492-in-the-a1-a2-linker-region-in-a-shear-and-glycan-dependent-manner-in-vitro
#20
Teresa M Brophy, Soracha E Ward, Thomas R McGimsey, Sonja Schneppenheim, Clive Drakeford, Jamie M O'Sullivan, Alain Chion, Ulrich Budde, James S O'Donnell
OBJECTIVE: Previous studies have demonstrated a role for plasmin in regulating plasma von Willebrand factor (VWF) multimer composition. Moreover, emerging data have shown that plasmin-induced cleavage of VWF is of particular importance in specific pathological states. Interestingly, plasmin has been successfully used as an alternative to ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif) in a mouse model of thrombotic thrombocytopenic purpura. Consequently, elucidating the molecular mechanisms through which plasmin binds and cleaves VWF is not only of basic scientific interest but also of direct clinical importance...
March 9, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
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