keyword
https://read.qxmd.com/read/36751751/detection-of-paroxysmal-nocturnal-haemoglobinuria-clones-in-cases-of-deep-vein-thrombosis-in-a-tertiary-care-centre-western-rajasthan
#21
JOURNAL ARTICLE
Siddhant Passey, Abhishek Purohit
BACKGROUND & OBJECTIVES: Paroxysmal nocturnal haemoglobinuria is a rare acquired disease characterized by bone marrow failure, intravascular haemolysis and thrombophilia. Thrombosis is the deadliest complication of paroxysmal nocturnal haemoglobinuria (PNH). The present study was conducted to study the prevalence of PNH in cases of deep vein thrombosis (DVT) which was previously undocumented from western Rajasthan. METHODS: In the present cross-sectional study, 61 adult patients with DVT were tested using flow cytometry to detect PNH clones...
September 2022: Indian Journal of Medical Research
https://read.qxmd.com/read/36703287/covid-induced-mesenteric-ischemia-in-a-patient-having-ulcerative-colitis-and-paroxysmal-nocturnal-haemoglobinuria-in-spite-of-thromboprophylaxis
#22
JOURNAL ARTICLE
Arup Choudhury, Mohan Sundaram, Thiyam Tozo Luwang, Harjeet Singh, Vishal Sharma
Inflammatory bowel disease and paroxysmal nocturnal hemoglobinuria (PNH) are both well-known prothrombotic states. However, ongoing thromboprophylaxis is usually effective in such conditions. However, we report about an imbalance that was triggered by like SARS-CoV-2 infection. There is evidence that SARS-CoV-2 infection leads to thrombosis of vessels. The thrombosis of mesenteric vessels can be multifocal and without respiratory symptoms and leads to devastating consequences like resection of large segments of the bowel and lifelong requirement of parenteral nutritional support...
January 26, 2023: Journal of the Royal College of Physicians of Edinburgh
https://read.qxmd.com/read/36669788/pseudo-thrombotic-microangiopathy-due-to-folate-deficiency
#23
JOURNAL ARTICLE
Emily Larkin, Samuel Konkol, Meghan Geraghty
Classically, deficiencies of vitamin B12 and folate are associated with megaloblastic anaemia. Additionally, vitamin B12 is able to cause a haemolytic anaemia in the form of pseudo-thrombotic microangiopathy (pseudo-TMA). Here, we present a case of a middle-aged woman with a history of Roux-en-Y gastric bypass who presented with dyspnoea and fatigue and was found to have thrombocytopenia and a non-immune haemolytic anaemia. Work-up for haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura, paroxysmal nocturnal haemoglobinuria, infection, malignancy and autoimmune conditions was unremarkable...
January 20, 2023: BMJ Case Reports
https://read.qxmd.com/read/36652996/furosemide-induced-haemolytic-anaemia-in-an-extreme-elderly-patient
#24
I-Wei Ho, Chin-Chou Huang
Furosemide, a loop diuretic, is commonly used to treat fluid overload symptoms and heart failure. Drug-induced immune haemolytic anaemia is an unusual drug-adverse event. Furosemide-induced haemolysis is even rarer. This case report presents a 91-year-old male who developed acute haemolytic anaemia 3 days after initiating furosemide to treat myocardial infarction complicated with acute decompensated heart failure. He had increased lactate dehydrogenase and unconjugated bilirubin with undetectable haptoglobin, which indicated the destruction of red blood cells...
January 18, 2023: ESC Heart Failure
https://read.qxmd.com/read/36608443/defected-lipid-rafts-suppress-cavin1-dependent-ifn-%C3%AE-signaling-endosome-in-paroxysmal-nocturnal-hemoglobinuria
#25
JOURNAL ARTICLE
Lijie Zeng, Hui Liu, Zhaoyun Liu, Liyan Li, Honglei Wang, Yingying Chen, Junshu Wu, Guanrou Wang, Lijuan Li, Rong Fu
Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal disorder of haematopoietic stem cells caused by somatic PIGA mutations, resulting in a deficiency in glycosylphosphatidylinositol-anchored proteins (GPI-AP). Some researchers uncovered that PNH cells displayed a GPI-mediated defect in lipid-raft formation. However, Lipid rafts play a crucial role in signaling, the signaling underlying lipid rafts in PNH have not yet been addressed. In this study, we reported that, IFN-α was significantly increased in PNH plasma compared with normal controls...
January 4, 2023: International Immunopharmacology
https://read.qxmd.com/read/36594332/paroxysmal-nocturnal-hemoglobinuria-in-systemic-lupus-erythematosus-a-rare-manifestation
#26
JOURNAL ARTICLE
M N Hasan, B I Rahman, M F Rahaman, S K Biswas, I Ahmed, M A Rahman
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare disorder of hematopoietic stem cells. The occurrence of PNH in a patient with systemic lupus erythematosus (SLE) is even rarer. One such presentation was seen in a 19 years old woman who presented with fever, multiple joint pain, photosensitivity, oral ulcer, hair loss and was diagnosed as a case of SLE and was admitted in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh on 7th February 2019. Subsequently she developed progressive anaemia and passing of dark colored urine...
January 2023: Mymensingh Medical Journal: MMJ
https://read.qxmd.com/read/36459381/pegcetacoplan-a-review-in-paroxysmal-nocturnal-haemoglobinuria
#27
REVIEW
Young-A Heo
Subcutaneous pegcetacoplan (EMPAVELI® in the USA and ASPAVELI® in the EU) is the first complement component 3 (C3) inhibitor approved for the treatment of adults with paroxysmal nocturnal haemoglobinuria (PNH) in the USA, and in adults with PNH who are anaemic after ≥ 3 months of treatment with a C5 inhibitor in the EU. In the phase III PRINCE trial in adults with PNH who were anaemic and naïve to a complement inhibitor therapy, pegcetacoplan was superior to the control group (supportive care, excluding complement inhibitors) in achieving haemoglobin stabilization and reducing lactate dehydrogenase levels...
December 2022: Drugs
https://read.qxmd.com/read/36396662/myelodysplastic-syndromes
#28
REVIEW
Huan Li, Fang Hu, Robert Peter Gale, Mikkael A Sekeres, Yang Liang
Myelodysplastic syndromes (MDS) are a family of myeloid cancers with diverse genotypes and phenotypes characterized by ineffective haematopoiesis and risk of transformation to acute myeloid leukaemia (AML). Some epidemiological data indicate that MDS incidence is increasing in resource-rich regions but this is controversial. Most MDS cases are caused by randomly acquired somatic mutations. In some patients, the phenotype and/or genotype of MDS overlaps with that of bone marrow failure disorders such as aplastic anaemia, paroxysmal nocturnal haemoglobinuria (PNH) and AML...
November 17, 2022: Nature Reviews. Disease Primers
https://read.qxmd.com/read/36394176/dose-optimalization-of-subcutaneous-ravulizumab-is-predicted-to-yield-significant-savings-and-to-improve-patient-friendliness
#29
JOURNAL ARTICLE
Mendy Ter Avest, Saskia M C Langemeijer, Nicole M A Blijlevens, Nicole C A J van de Kar, Rob Ter Heine
Ravulizumab is an expensive complement C5-inhibitor for the treatment of paroxysmal nocturnal haemoglobinuria. Recently, a subcutaneous formulation has entered the market, for which the approved dosing regimen results in supratherapeutic ravulizumab concentrations in the majority of patients in the registration studies. Therefore, we explored alternative dosing regimens in silico based on the registration data of the manufacturer. Extending the interval from 1 to 2 weeks or individualized dosing based on therapeutic drug monitoring resulted in therapeutic ravulizumab concentrations and comparable predicted efficacy in terms of lactate dehydrogenase normalization, with dose reductions up to 64%...
March 2023: British Journal of Clinical Pharmacology
https://read.qxmd.com/read/36255008/detection-of-paroxysmal-nocturnal-haemoglobinuria-clones-in-cases-of-deep-vein-thrombosis-in-a-tertiary-care-centre-western-rajasthan
#30
JOURNAL ARTICLE
Siddhant Passey, Abhishek Purohit
BACKGROUND & OBJECTIVES: Paroxysmal nocturnal haemoglobinuria is a rare acquired disease characterized by bone marrow failure, intravascular haemolysis and thrombophilia. Thrombosis is the deadliest complication of paroxysmal nocturnal haemoglobinuria (PNH). The present study was conducted to study the prevalence of PNH in cases of deep vein thrombosis (DVT) which was previously undocumented from western Rajasthan. METHODS: In the present cross-sectional study, 61 adult patients with DVT were tested using flow cytometry to detect PNH clones...
October 18, 2022: Indian Journal of Medical Research
https://read.qxmd.com/read/36165770/the-burden-of-illness-of-patients-with-paroxysmal-nocturnal-haemoglobinuria-receiving-c5-inhibitors-clinical-outcomes-and-medical-encounters-from-the-patient-perspective
#31
JOURNAL ARTICLE
Flore Sicre de Fontbrune, Pascale Burmester, Maria Piggin, Joana E Matos, Halley Costantino, Koo Wilson, Zalmai Hakimi, Jameel Nazir, Renaud Desgraz, Jesse Fishman, Emmelie Persson, Jens Panse
OBJECTIVES: To assess the clinical and healthcare resource burden among C5 inhibitor (C5i)-treated patients with paroxysmal nocturnal haemoglobinuria (PNH), using patient-reported data. METHODS: This web-based, cross-sectional survey (01FEB2021-31MAR2021) of adults with PNH treated with eculizumab (France, Germany, UK) or ravulizumab (Germany) included: patient characteristics; treatment patterns/dosage; haematological outcomes (haemoglobin [Hb] levels, transfusions, thrombotic events, breakthrough haemolysis); and medical encounters...
December 2022: Hematology (Amsterdam, Netherlands)
https://read.qxmd.com/read/36143962/pancytopenia-in-a-case-of-aplastic-anaemia-paroxysmal-nocturnal-haemoglobinuria-unmasked-by-sars-cov-2-infection-a-case-report
#32
Arcangelo Iannuzzi, Antonio Parrella, Francesca De Ritis, Anna Cammarota, Lucia Berloco, Francesca Paudice, Giovanni D'Angelo, Emilio Aliberti, Gabriella Iannuzzo
During an acute SARS-CoV-2 infection, a diagnosis of Aplastic Anaemia associated with Paroxysmal Nocturnal Haemoglobinuria (AA/PNH) was made in a 78-year-old woman who had presented to the emergency department with severe pancytopenia. It is possible that she had subclinical AA/PNH that was unmasked during the acute COVID-19 infection, but we can also suspect a direct role of the virus in the pathogenesis of the disease, or we can hypothesize that COVID-19 infection changed the phosphatidylinositol glycan class A (PIGA) gene pathway...
September 15, 2022: Medicina
https://read.qxmd.com/read/36116096/the-correlation-between-multiple-congenital-anomalies-hypotonia-seizures-syndrome-2-and-piga-a-case-of-novel-piga-germline-variant-and-literature-review
#33
JOURNAL ARTICLE
Xiangyu Liu, Jing Meng, Jinhui Ma, Jianbo Shu, Chunyu Gu, Xiaofang Chen, Dong Li, Chunquan Cai
BACKGROUND: PIGA (PIG class A) gene codes for the PIG-A protein, which is a catalytic subunit of GPI-GlcNAc transferase. GPI-anchored proteins play an important role in the metabolism of mammals. Somatic variants of PIGA genes in bone marrow hematopoietic stem cells often result in paroxysmal nocturnal haemoglobinuria, and the germline PIGA variants cause multiple congenital anomalies hypotonia seizures syndrome 2 (MCAHS2) because of glycosylphosphatidylinositol metabolic abnormalities...
September 18, 2022: Molecular Biology Reports
https://read.qxmd.com/read/36102159/whole-blood-transfusion-and-paroxysmal-nocturnal-haemoglobinuria-meet-again-minor-incompatibility-major-trouble
#34
JOURNAL ARTICLE
Ingvild Jenssen Laegreid, Thomas Wilson, Kristoffer Hjertø Naess, Siw Leiknes Ernstsen, Vibeke Schou, Mirjana Grujic Arsenovic
BACKGROUND AND OBJECTIVES: The field of transfusion medicine started out with whole blood transfusion to treat severe anaemia and other deficiencies, and then transitioned to component therapy, largely leaving the practice, and experiences, of whole blood transfusions behind. Currently, the field is circling back and whole blood is gaining ground as an alternative to massive transfusion protocols. MATERIALS AND METHODS: Herein we describe a severely anaemic paroxysmal nocturnal haemoglobinuria (PNH) patient initially suspected of suffering from renal haemorrhage, receiving a standard low-titre group O whole blood transfusion during pre-hospital transportation...
September 14, 2022: Vox Sanguinis
https://read.qxmd.com/read/36100921/correction-pulmonary-embolism-in-a-patient-with-eltrombopag-treated-aplastic-anaemia-and-paroxysmal-nocturnal-haemoglobinuria-clone-during-covid-19-pneumonia
#35
Alessandro Bosi, Wilma Barcellini, Bruno Fattizo
No abstract text is available yet for this article.
September 13, 2022: Thrombosis Journal
https://read.qxmd.com/read/36055332/pegcetacoplan-versus-eculizumab-in-patients-with-paroxysmal-nocturnal-haemoglobinuria-pegasus-48-week-follow-up-of-a-randomised-open-label-phase-3-active-comparator-controlled-trial
#36
RANDOMIZED CONTROLLED TRIAL
Régis Peffault de Latour, Jeff Szer, Ilene C Weitz, Alexander Röth, Britta Höchsmann, Jens Panse, Kensuke Usuki, Morag Griffin, Jean-Jacques Kiladjian, Carlos M de Castro, Hisakazu Nishimori, Temitayo Ajayi, Mohammed Al-Adhami, Pascal Deschatelets, Cedric Francois, Federico Grossi, Antonio M Risitano, Peter Hillmen
BACKGROUND: In the PEGASUS trial, the complement C3 inhibitor, pegcetacoplan, showed superiority to eculizumab in improving haematological outcomes in adult patients with paroxysmal nocturnal haemoglobinuria and suboptimal response to eculizumab at 16 weeks. The aim of the open-label period was to evaluate the long-term efficacy and safety of pegcetacoplan through to 48 weeks. METHODS: PEGASUS was a phase 3, randomised, open-label, active-comparator controlled trial conducted in 44 centres in Australia, Belgium, Canada, France, Germany, Japan, Russia, South Korea, Spain, the UK, and the USA...
September 2022: Lancet Haematology
https://read.qxmd.com/read/35999631/pulmonary-embolism-in-a-patient-with-eltrombopag-treated-aplastic-anaemia-and-paroxysmal-nocturnal-haemoglobinuria-clone-during-covid-19-pneumonia
#37
JOURNAL ARTICLE
Bosi Alessandro, Barcellini Wilma, Fattizzo Bruno
Thrombosis in patients with thrombocytopenia has several risk factors, both disease-related and treatment-associated. Recently, COVID-19 infection was recognized as an additional risk factor, further complicating the delicate balance between thrombosis and bleeding in these patients. Here we describe the case of a patient with aplastic anaemia on eltrombopag who developed pulmonary embolism during COVID-19 pneumonia, despite receiving oral anticoagulation with edoxaban. Notably, he was also carrying a large paroxysmal nocturnal haemoglobinuria clone, although without evidence of haemolysis...
August 24, 2022: Thrombosis Journal
https://read.qxmd.com/read/35971099/generalized-pulp-canal-obliteration-in-a-patient-on-long-term-glucocorticoids-a-case-report-and-literature-review
#38
REVIEW
Ban Jiandong, Zhang Yunxiao, Wang Zuhua, Hou Yan, Geng Shuangshuang, Li Junke, Wang Hongwei, Xu Hua
BACKGROUND: The calcification of the tooth pulp is a pathological condition that occurs in response to various factors. A uncommon haematological condition known as paroxysmal nocturnal haemoglobinuria (PNH) is characterized by bouts of haemolysis, and it requires long-term use of glucocorticoids (GCs). CASE PRESENTATION: A female patient who was diagnosed with PNH and had a history of long-term use of GCs came to our department for root canal therapy (RCT) for teeth 25, 26, and 27...
August 15, 2022: BMC Oral Health
https://read.qxmd.com/read/35941884/severe-aplastic-anaemia-after-serial-vaccinations-for-sars-cov-2-pneumococcus-and-seasonal-influenza
#39
Xiao Wang, Dorottya Laczko, Gabriel C Caponetti, Susan Rabatin, Daria V Babushok
We present a 67-year-old woman who developed progressive pancytopenia over 10 months, concomitant with administration of severe adult respiratory syndrome coronavirus-2 (SARS-CoV-2), pneumococcal and influenza vaccines. She developed mild leukopenia ∼2 weeks after the SARS-CoV-2 mRNA vaccine sequence, with progressive symptoms after subsequent vaccines, eventually developing severe aplastic anaemia (SAA). While there have been several reports of vaccine-related SAA, at time of submission, our case is the first reported to develop after the Moderna mRNA SARS-CoV-2 vaccine, as well as the first to document the gradual development of SAA over the course of many vaccine exposures...
August 2022: EJHaem
https://read.qxmd.com/read/35848629/breakthrough-haemolysis-in-paroxysmal-nocturnal-haemoglobinuria-after-covid-19-infection-and-covid-vaccination-what-is-worse
#40
JOURNAL ARTICLE
Francesca Cavallaro, Maria C Pasquini, Juri A Giannotta, Federica Cattina, Wilma Barcellini, Bruno Fattizzo
No abstract text is available yet for this article.
May 19, 2022: Blood Transfusion
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