Microangiopathic Hemolytic Anemia Research This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Five years of caplacizumab - Lessons learned and remaining controversies in immune-mediated thrombotic thrombocytopenic purpura
J Thromb Haemost. 2023 Aug 8:S1538-7836(23)00591-3. doi: 10.1016/j.jtha.2023.07.027. Online ahead of print.ABSTRACTImmune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare hematologic disease caused by autoantibodies against ADAMTS13 that trigger a microangiopathic hemolytic anemia. Therapeutic plasma exchange and glucocorticoids have been the mainstay of treatment for the past 30 years. In 2019, caplacizumab was approved as an addition to this regimen for the acute treatment of iTTP. Randomized controlled trials and real-world evidence have shown that caplacizumab reduces the time to platelet count normalizati...
Source: Thrombosis and Haemostasis - August 10, 2023 Category: Hematology Authors: Linus Alexander Volker Paul Thomas Brinkkoetter Spero R Cataland Camila Masias Source Type: research

Genotypic analysis of a large cohort of patients with suspected atypical hemolytic uremic syndrome
J Mol Med (Berl). 2023 Jul 19. doi: 10.1007/s00109-023-02341-4. Online ahead of print.ABSTRACTAtypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Complement and coagulation gene variants have been associated with aHUS susceptibility. We assessed the diagnostic yield of a next-generation sequencing (NGS) panel in a large cohort of Canadian patients with suspected aHUS. Molecular testing was performed on peripheral blood DNA samples from 167 patients, collected between May 2019 and December 2021, using a clinically validated NGS pipeline. Co...
Source: Molecular Medicine - July 19, 2023 Category: Molecular Biology Authors: Dervla M Connaughton Pratibha Bhai Paul Isenring Mohammed Mahdi Bekim Sadikovic Laila C Schenkel Source Type: research

Genotypic analysis of a large cohort of patients with suspected atypical hemolytic uremic syndrome
AbstractAtypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Complement and coagulation gene variants have been associated with aHUS susceptibility. We assessed the diagnostic yield of a next-generation sequencing (NGS) panel in a large cohort of Canadian patients with suspected aHUS. Molecular testing was performed on peripheral blood DNA samples from 167 patients, collected between May 2019 and December 2021, using a clinically validated NGS pipeline. Coding exons with 20 base pairs of flanking intronic regions for 21 aHUS-associated or c...
Source: Journal of Molecular Medicine - July 19, 2023 Category: Molecular Biology Source Type: research

Epidemiology and clinical features of Hypnale nepa (hump-nosed pit viper) envenoming in Sri Lanka
The objectives of this study were to describe epidemiological and clinical features of H. nepa bites. A prospective observational study was conducted for patients admitted with H. nepa bites to Teaching Hospital, Ratnapura, Sri Lanka for 5 years commencing from June 2015. Species identification was done using a standard key. There were 14 (3.6%) patients with H. nepa bites of which 9 (64%) were males and 5 (36%) were females. Their age ranged from 20 to 73 years (median 37.5). Seven bites (50%) occurred on lower limbs. Majority of bites (10; 71%) happened at daytime [0600-1759 h] in tea estates (8; 57%). Most patients (8; ...
Source: Toxicon - June 15, 2023 Category: Toxicology Authors: R M M K Namal Rathnayaka P E Anusha Nishanthi Ranathunga S A M Kularatne Source Type: research

Safety and efficacy of caplacizumab retreatment in a real-life monocentric cohort of patients with immune-mediated thrombotic thrombocytopenic purpura
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is an acute life-threatening disease characterized by the association of thrombocytopenia, microangiopathic hemolytic anemia, organ involvement and ADAMTS-13 protease deficiency [1]. The standard of care for iTTP has historically been based on therapeutic plasma exchange (TPE) and immune suppression. More recently, the treatment with caplacizumab has led to a significantly faster iTTP recovery. This is a nanobody that targets the interaction between platelet's glycoprotein 1b and the ultra-large A1 domain of von Willebrand factor (VWF). (Source: Thrombosis Research)
Source: Thrombosis Research - June 15, 2023 Category: Hematology Authors: G. Caocci, O. Mulas, D. Mantovani, N. Bandinu, G. La Nasa Tags: Letter to the Editors-in-Chief Source Type: research

Potential modifications of the PLASMIC scoring system for predicting thrombotic thrombocytopenic purpura: Sometimes, less is more
CONCLUSIONS: Based on the results of this validation study, removing MCV from the PLASMIC score led to the categorization of eight non-TTP cases in the low-risk category, and this could avoid unnecessary plasma exchange. However, in our study increasing the specificity was at the expense of the sensitivity by missing one patient with this new scoring system without MCV. Further multicenter studies with large sample sizes are required owing to the fact that different parameters may be effective in TTP prediction among different populations.PMID:37309683 | DOI:10.1111/ijlh.14115 (Source: International Journal of Laboratory Hematology)
Source: International Journal of Laboratory Hematology - June 13, 2023 Category: Hematology Authors: Bedrettin Orhan Vildan Özkocaman Çiğdem Akdemir Tuba Ersal İbrahim Ethem Pınar Cumali Yal çın Ömer Candar Sinem Çubukçu Tuba G üllü Koca P ınar Ambarcıoğlu R ıdvan Ali Fahir Özkalemkaş Source Type: research

Necrotizing pancreatitis, microangiopathic hemolytic anemia and thrombocytopenia following the second dose of Pfizer/BioNTech COVID-19 mRNA vaccine
We report a 31-year-old female with a history of borderline personality disorder, intravenous drug abuse, allergic asthma, eating disorder, psoriatic arthritis treated with tofacitinib, neurogenic bladder disturbance, cholecystectomy, recurrent thoracic herpes zoster, vaginal candida infections and urinary tract infections, who developed pancreatitis associated with thrombotic microangiopathy and hemolytic-uremic syndrome 10 days after the second vaccination, whereas the first has been well tolerated. She was treated by plasma exchange, and eventually by transgastric drainage with implantation of a plastic stent to remove ...
Source: Herpes - June 6, 2023 Category: Infectious Diseases Authors: Claudia St öllberger Kastriot Kastrati Clemens Dejaco Martina Scharitzer Josef Finsterer Patrick Bugingo Madeleine Melichart-Kotik Astrid Wilfing Source Type: research

Necrotizing pancreatitis, microangiopathic hemolytic anemia and thrombocytopenia following the second dose of Pfizer/BioNTech COVID-19 mRNA vaccine
We report a 31-year-old female with a history of borderline personality disorder, intravenous drug abuse, allergic asthma, eating disorder, psoriatic arthritis treated with tofacitinib, neurogenic bladder disturbance, cholecystectomy, recurrent thoracic herpes zoster, vaginal candida infections and urinary tract infections, who developed pancreatitis associated with thrombotic microangiopathy and hemolytic-uremic syndrome 10 days after the second vaccination, whereas the first has been well tolerated. She was treated by plasma exchange, and eventually by transgastric drainage with implantation of a plastic stent to remove ...
Source: Wiener Klinische Wochenschrift - June 6, 2023 Category: General Medicine Authors: Claudia St öllberger Kastriot Kastrati Clemens Dejaco Martina Scharitzer Josef Finsterer Patrick Bugingo Madeleine Melichart-Kotik Astrid Wilfing Source Type: research

Clinical characteristics of anti-GBM disease with thrombotic microangiopathy: a case report and literature review
AbstractThe coexistence of anti-glomerular basement membrane (anti-GBM) disease with thrombotic microangiopathy (TMA) is rarely encountered, and the clinical characteristics of this phenomenon are not well known.A 76-year-old Japanese woman with a history of idiopathic pulmonary disease was diagnosed with anti-GBM disease due to rapidly progressive glomerulonephritis and a positive anti-GBM antibody test result. We treated the patient with hemodialysis, glucocorticoids, and plasmapheresis. During treatment, the patient suddenly became comatose. TMA was then diagnosed because of thrombocytopenia and microangiopathic hemolyt...
Source: CEN Case Reports - May 22, 2023 Category: Urology & Nephrology Source Type: research

Congenital Thrombotic Thrombocytopenic Purpura Masquerading As Vitamin B12 Deficiency
Congenital thrombotic thrombocytopenic purpura (CTTP), also called Upshaw-Schulman syndrome (USS), is a rare autosomal recessive disorder resulting from the deficiency of the ADAMTS13. CTTP is characterized by the formation of platelet-rich thrombi in small vessels of multiple organs, resulting in thrombocytopenia and microangiopathic hemolytic anemia, eventually leading to organ failure. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - May 15, 2023 Category: Hematology Authors: Deedar Nanjiani, Sohail Kumar, Nitik Sharma, Sundus Nasim, Dua Azim, Oam Parkash Source Type: research

A case of infection-induced thrombotic thrombocytopenic purpura
CONCLUSION: TTP is an acute severe disease with complex etiology, abrupt onset, and dangerous conditions. In this patient with TTP, an important cause of the disease may have been an acute gastrointestinal infection. The plasma examination in another hospital revealed positive results for ADAMTS13 inhibitors, providing strong evidence for the diagnosis of this case. Multiple plasma exchanges and glucocorticoids yielded favorable treatment results and were critical measures of successful treatment of TTP.PMID:37182863 | DOI:10.3233/CH-231784 (Source: Clinical Hemorheology and Microcirculation)
Source: Clinical Hemorheology and Microcirculation - May 14, 2023 Category: Hematology Authors: Shaohua Cui Chaoyue Liang Lixia Geng Source Type: research