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Thrombotic thrombocytopenic purpura

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https://www.readbyqxmd.com/read/29674502/dissecting-the-pathophysiology-of-immune-thrombotic-thrombocytopenic-purpura-interplay-between-genes-and-environmental-triggers
#1
Johana Hrdinová, Silvia D'Angelo, Nuno A G Graça, Bogac Ercig, Karen Vanhoorelbeke, Agnès Veyradier, Jan Voorberg, Paul Coppo
Although outstanding progress has been made in comprehending the pathophysiology of thrombotic thrombocytopenic purpura, our understanding of the immunopathogenesis of the disease is only in its earlier stage. Anti-ADAMTS13 auto-antibodies were shown to block proteolysis of von Willebrand factor and/or induce ADAMTS13 clearance from the circulation. However, which immune cells are involved in the production of anti-ADAMTS13 autoantibodies and hence account for the remarkable efficacy of the B-cell depleting agents in this disease, remain to be identified...
April 19, 2018: Haematologica
https://www.readbyqxmd.com/read/29674195/thrombotic-microangiopathies-of-pregnancy-differential-diagnosis
#2
REVIEW
M Gupta, B B Feinberg, R M Burwick
Thrombotic microangiopathy (TMA) disorders are characterized by microangiopathic hemolytic anemia, thrombocytopenia and end-organ injury. In pregnancy and postpartum, TMA is most commonly encountered with HELLP (hemolysis, elevated liver enzymes, low platelet count syndrome) or preeclampsia with severe features, but rarely TMA is due to thrombotic thrombocytopenic purpura (TTP) or atypical hemolytic uremic syndrome (aHUS). Due to overlapping clinical and laboratory features, TTP and aHUS are often mistaken for preeclampsia or HELLP...
February 16, 2018: Pregnancy Hypertension
https://www.readbyqxmd.com/read/29659973/the-diagnostic-challenge-of-acquired-thrombotic-thrombocytopenic-purpura-in-children-case-report-and-review-of-the-literature
#3
Elise Schapkaitz, Tanya Marié Schickerling
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare disease in children. Despite advances in diagnosis and treatment, acquired TTP remains a challenging disease due to the lack of a simple diagnostic test and the variable response to plasma exchange. Herein, we describe a case of a 5-year-old of black ethnicity boy who presented with a sudden onset of fatigue and body aches. Laboratory investigations revealed a thrombotic microangiopathic manifestation. TTP was diagnosed, and plasma exchange and corticosteroids were initiated, with an excellent response...
April 12, 2018: Laboratory Medicine
https://www.readbyqxmd.com/read/29657197/adamts-13-level-in-children-with-severe-diarrhea-associated-hemolytic-uremic-syndrome-unmasking-new-association
#4
Naglaa A Khalifa, Heba H Gawish, Noora A Khalifa, Doaa M Tawfeek, Saed M Morsy
Severe deficiency of ADAMTS-13 leads to thrombotic thrombocytopenic purpura. Few studies have reported reduced activity of ADAMTS-13 in patients with atypical and typical hemolytic uremic syndrome (HUS). We hypothesized that ADAMTS-13 deficiency might play a role in the pathogenesis of severe HUS. This study aimed to evaluate the ADAMTS-13 level in severe typical HUS. This prospective case-control study was carried out in the Pediatric Nephrology Unit and Clinical Pathology Department, Faculty of Medicine, Zagazig University from February 2013 to February 2014...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657193/atypical-hemolytic-uremic-syndrome-laboratory-characteristics-complement-amplifying-conditions-renal-biopsy-and-genetic-mutations
#5
REVIEW
Mohammad A Hossain, Anmol Cheema, Sheila Kalathil, Ravneet Bajwa, Eric J Costanzo, James Cosentino, Jennifer Cheng, Tushar Vachharajani, Arif Asif
Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and widespread damage to multiple organs including the kidney. The syndrome has a high mortality necessitating the need for an early diagnosis to limit target organ damage. Because thrombotic microangiopathies present with similar clinical picture, accurate diagnosis of aHUS continues to pose a diagnostic challenge. This article focuses on the role of four distinct aspects of aHUS that assist clinicians in making an accurate diagnosis of aHUS...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29643888/spectrum-of-complications-of-severe-dka-in-children-in-pediatric-intensive-care-unit
#6
Qalab Abbas, Saba Arbab, Anwar Ul Haque, Khadija Nuzhat Humayun
Objectives: To describe the spectrum of complications of Diabetic Ketoacidosis (DKA) observed in children admitted with severe DKA. Methods: Retrospective review of the medical records of all children admitted with the diagnosis of severe DKA in Pediatric Intensive Care Unit (PICU) of the Aga Khan University Hospital, from January 2010 to December 2015 was done. Data was collected on a structured proforma and descriptive statistics were applied. Results: Total 37 children were admitted with complicated DKA (1...
January 2018: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/29621007/disseminated-intravascular-coagulation-is-it-fact-or-fancy
#7
Jae C Chang
: 'Disseminated intravascular coagulation (DIC)' occurs commonly in critical illnesses such as sepsis, trauma, cancer, and complications of surgery and pregnancy. Mortality is very high. The pathogenesis has been ascribed to tissue factor-initiated coagulation disorder, resulting in disseminated microblood clots that are made of platelets, plasma factors, fibrins, and blood cells. True DIC depletes coagulation factors and consumes platelets, and activates fibrinolysis. 'DIC' is assumed to orchestrate thrombocytopenia, microangiopathic hemolytic anemia and hypoxic multiorgan dysfunction syndrome, and causes hemorrhagic disorder due to depleted coagulation factors...
April 4, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29593914/rituximab-induced-pulmonary-edema-managed-with-extracorporeal-life-support
#8
Jacob R Miller, Warren Isakow, Muhammad F Masood, Patrick Aguilar, Kristen M Sanfilippo, Keki R Balsara, Akinobu Itoh
Though rare, rituximab has been reported to induce severe pulmonary edema. We describe the first report of ECLS utilization for this indication. A 31-year-old female with severe thrombotic thrombocytopenic purpura developed florid pulmonary edema after rituximab infusion. Despite advanced ventilatory settings, she developed severe respiratory acidosis and remained hypoxemic with a significant vasopressor requirement. Since her pulmonary insult was likely transient, ECLS was considered. Due to combined cardiorespiratory failure, she received support with peripheral venoarterial ECLS...
2018: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29588177/a-43-year-old-woman-with-unexplained-elevation-of-hcg
#9
Lisa M Johnson, Thomas J Gniadek, Claudia S Cohn, Gary Bachowski, Amy B Karger
OBJECTIVE: This case report investigates an unusual hCG result in a woman who is not pregnant. PATIENT AND METHODS: A 43-year-old woman was admitted for recurrence of thrombotic thrombocytopenic purpura (TTP) and therapeutic plasma exchange (TPE) was initiated. Prior to transitioning the patient from TPE to immunosuppressive therapy, a serum qualitative hCG test was performed and was positive. Several etiologies for elevated hCG were considered and investigated, including heterophile antibody interference, endogenous hCG from pituitary or malignancy, and exogenous hCG...
March 26, 2018: Clinical Biochemistry
https://www.readbyqxmd.com/read/29582550/consensus-opinion-on-diagnosis-and-management-of-thrombotic-microangiopathy-in-australia-and-new-zealand
#10
REVIEW
Lucy C Fox, Solomon J Cohney, Joshua Y Kausman, Jake Shortt, Peter D Hughes, Erica M Wood, Nicole M Isbel, Theo de Malmanche, Anne Durkan, Pravin Hissaria, Piers Blombery, Thomas D Barbour
Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film. Whilst TMA of any aetiology generally demands prompt treatment, this is especially so in thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), where organ failure may be precipitous, irreversible and fatal...
March 27, 2018: Internal Medicine Journal
https://www.readbyqxmd.com/read/29572925/correlating-s100b-with-disease-course-in-a-case-of-new-onset-acquired-thrombotic-thrombocytopenic-purpura-ttp-could-this-be-a-new-predictive-biomarker-in-ttp
#11
Patrick Kuhlman, Peter Miller, Andrew Farland, John Owen, Katharine Batt
Acute thrombotic thrombocytopenic purpura (TTP) is an aggressive thrombotic microangiopathy that if not treated, can have a 90% mortality rate. Clinical manifestations of this disease include profound thrombocytopenia, hemolytic anemia, and end-organ dysfunction. Neurologic symptoms can occur in 80% of patients and range from mild confusion to coma (Scully et al., Br J Haematol 142:819-826). Here, we present the clinical course of a patient diagnosed with new onset acquired TTP who presented with neurologic changes that waxed and waned during her disease course...
March 23, 2018: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/29567779/mass-spectrometry-assisted-identification-of-adamts13-derived-peptides-presented-on-hla-dr-and-hla-dq
#12
Johana Hrdinová, Fabian C Verbij, Paul H P Kaijen, Robin B Hartholt, Floris van Alphen, Neubury Lardy, Anja Ten Brinke, Karen Vanhoorelbeke, Pooja J Hindocha, Anne S De Groot, Alexander B Meijer, Jan Voorberg, Ivan Peyron
Formation of microthrombi is a hallmark of acquired thrombotic thrombocytopenic purpura. These microthrombi originate from insufficient processing of ultra large von Willebrand factor multimers by ADAMTS13 due to the development of anti-ADAMTS13 autoantibodies. Several studies have identified the major histocompatibility complex class II alleles HLA-DRB1*11, HLA-DQB1*03 and HLA-DQB1*02:02 as risk factors for acquired thrombotic thrombocytopenic purpura development. Previous research in our department indicated that ADAMTS13 CUB2 domain-derived peptides FINVAPHAR and LIRDTHSLR are presented on HLA-DRB1*11 and HLA-DRB1*03, respectively...
March 22, 2018: Haematologica
https://www.readbyqxmd.com/read/29567368/efficacy-of-eculizumab-in-severe-adamts13-deficient-thrombotic-thrombocytopenic-purpura-ttp-refractory-to-standard-therapies
#13
Ernesto Vigna, Annamaria Petrungaro, Anna Perri, Dario Terzi, Anna Grazia Recchia, Francesco Mendicino, Antonella La Russa, Sabrina Bossio, Laura De Stefano, Francesco Zinno, Renzo Bonofiglio, Fortunato Morabito, Massimo Gentile
Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathic hemolytic anemia (MAHA) defined by mechanical hemolytic anemia, severe thrombocytopenia, and systemic visceral ischemia due to systemic platelet-rich microthrombi. Forty percent of patients with autoimmune TTP experience one or multiple relapses. Patients with refractory TTP are currently managed by corticosteroids, twice-daily PEX, and the anti-CD20 monoclonal antibody rituximab. Herein, we report two cases of severe TTP, refractory to those standard agents...
March 15, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29564686/ofatumumab-for-acute-treatment-and-prophylaxis-of-a-patient-with-multiple-relapses-of-acquired-thrombotic-thrombocytopenic-purpura
#14
Hanny Al-Samkari, Rachael F Grace, Jean M Connors
Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder resulting in potentially life-threating systemic thrombotic microangiopathy due to production of antibodies directed against the von Willebrand factor-cleaving protease ADAMTS13. Typically managed with plasma exchange, glucocorticoids, and the first-generation anti-CD20 monoclonal antibody rituximab, patients with multiple relapses or refractory disease present unique management challenges. We describe a case of a young woman with multiple relapses of TTP despite standard therapy who was treated with ofatumumab, a second-generation anti-CD20 monoclonal antibody, after developing a severe hypersensitivity reaction to rituximab precluding its use...
March 21, 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29563339/germline-mutations-in-the-alternative-pathway-of-complement-predispose-to-hellp-syndrome
#15
Arthur J Vaught, Evan M Braunstein, Jagar Jasem, Xuan Yuan, Igor Makhlin, Solange Eloundou, Andrea C Baines, Samuel A Merrill, Shruti Chaturvedi, Karin Blakemore, C John Sperati, Robert A Brodsky
BACKGROUND: HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome is a severe variant of hypertensive disorders of pregnancy affecting approximately 1% of all pregnancies, and has significant maternal and fetal morbidity. Previously, we showed that upregulation of the alternative pathway of complement (APC) plays a role in HELLP syndrome. We hypothesize that HELLP syndrome follows a 2-hit disease model similar to atypical hemolytic uremic syndrome (aHUS), requiring both genetic susceptibility and an environmental risk factor...
March 22, 2018: JCI Insight
https://www.readbyqxmd.com/read/29550758/thrombotic-microangiopathy-associated-with-mycoplasma-pneumoniae-infection
#16
Filipa Caeiro Alves, Rute Aguiar, Pedro Pessegueiro, Carlos Pires
Thrombotic microangiopathy (TMA) comprises a process of sequential endothelial damage, microvascular thrombosis, consumptive thrombocytopenia and microangiopathic haemolytic anaemia that can affect several organs, including the kidney. A 36-year-old woman was presented with a petechial rash 3 weeks after an upper respiratory tract infection. Laboratory results showed normocytic normochromic anaemia, thrombocytopenia and evidence of TMA with decreased haptoglobin, elevated serum lactate dehydrogenase and a peripheral blood smear with numerous schistocytes...
March 17, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29531931/thrombotic-thrombocytopenic-purpura-in-a-child-with-diabetic-ketoacidosis
#17
Neda Mostofizadeh, Serajaddin Arefnia, Mahin Hashemipour, Elham Hashemi Dehkordi
Thrombotic thrombocytopenic purpura (TTP) secondary to diabetic ketoacidosis has been rarely reported and is considered as a rare complication. If left untreated, this condition could be life threatening with considerable morbidity and mortality. Herein, we report a 6-year-old girl with reduced consciousness and respiratory distress with a history of polydipsia and polyuria in the 2 weeks before hospitalization. The patient was initially diagnosed as diabetic ketoacidosis based on clinical and laboratory findings and treated accordingly...
2018: Advanced Biomedical Research
https://www.readbyqxmd.com/read/29523490/thrombotic-microangiopathy-in-a-very-young-infant-with-mitral-valvuloplasty
#18
Yuka Matsunaga, Masataka Ishimura, Hazumu Nagata, Kiyoshi Uike, Tadamune Kinjo, Masayuki Ochiai, Kenichiro Yamamura, Hidetoshi Takada, Yoshihisa Tanoue, Masaki Hayakawa, Masanori Matsumoto, Toshiro Hara, Shouichi Ohga
BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle...
February 7, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29512619/a-practical-and-case-based-approach-to-thrombocytopenia-in-cardiology-practice
#19
Erman Öztürk, Ferit Onur Mutluer
In cardiology practice, anticoagulation and antiplatelet therapies are essential for most patients. As of yet, there is no high quality evidence regarding these treatments in thrombocytopenic patients, which continues to be an issue. Thrombocytopenia is defined as a platelet count of <150x109/L and is classified as severe when the platelet count is <50x109/L. Pseudothrombocytopenia, drug-induced thrombocytopenia, immune thrombocytopenia, heparin-induced thrombocytopenia, and thrombotic thrombocytopenic purpura are some of the main causes of thrombocytopenia...
March 2018: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/29503132/a-multicenter-experience-of-thrombotic-microangiopathies-in-turkey-the-turkish-hematology-research-and-education-group-threg-tma01-study
#20
Emre Tekgündüz, Mehmet Yılmaz, Mehmet Ali Erkurt, Ilhami Kiki, Ali Hakan Kaya, Leylagul Kaynar, Inci Alacacioglu, Guven Cetin, Ibrahim Ozarslan, Irfan Kuku, Gulden Sincan, Ozan Salim, Sinem Namdaroglu, Abdullah Karakus, Volkan Karakus, Fevzi Altuntas, Ismail Sari, Gulsum Ozet, Ismet Aydogdu, Vahap Okan, Emin Kaya, Rahsan Yildirim, Esra Yildizhan, Gokhan Ozgur, Osman Ilhami Ozcebe, Bahriye Payzin, Seval Akpinar, Fatih Demirkan
Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission...
February 20, 2018: Transfusion and Apheresis Science
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