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JOURNAL ARTICLE
Francis Ayuk, Eva-Maria Wagner-Drouet, Daniel Wolff, Natascha von Huenerbein, Ute-Marie von Pein, Evgeny Klyuchnikov, Stephanie von Harsdorf, Christian Koenecke, Herbert Sayer, Nicolaus Kröger
Although most patients with chronic graft-versus-host disease (cGVHD) show initial response to first-line therapy, long-term clinically meaningful success of first-line treatment remains rare. In a prospective multicentre phase II trial in 6 German centers, patients with newly diagnosed moderate or severe cGVHD received prednisone and everolimus for 12 months followed by a 1-year follow-up period. Primary endpoint was treatment success (TS) at 6 months defined as patient being alive, achieving PR or CR of cGVHD, having no relapse of underlying disease and requiring no secondary treatment for cGVHD...
May 2, 2024: Bone Marrow Transplantation
#2
Madeeha Subhan Waleed, Lohitha Dhulipalla, Muhammad Niazi, Terenig Terjanian, Meekoo Dhar
Thrombotic thrombocytopenic purpura (TTP) is a rare disease that is part of a vast spectrum of thrombotic microangiopathies (TMAs). Despite the rarity of TTP, clinicians must maintain a high suspicion of this disease. The condition is characterized by fever, low platelets, hemolytic anemia, renal abnormalities, and neurological dysfunction. However, all these symptoms are not necessarily present in all the patients. In this review, we describe a case of a 51-year-old female who presented to the emergency department (ED) with chief complaints of dizziness and lightheadedness, subsequently leading to a diagnosis of TTP, caused as a result of COVID-19...
March 2024: Curēus
#3
JOURNAL ARTICLE
Ping L Zhang, Brandon D Metcalf, Sarang Khan, Jamal Abukhaled, Khalid Zafar, Wei Li, Hassan D Kanaan
CONTEXT: IgM-dominant immune complex-mediated glomerulonephritis (IgM-dominant ICMGN) is a rare renal entity, characterized by a membranoproliferative pattern by light microscopy, dominant IgM staining by immunofluorescent staining, and subendothelial deposits by electron microscopy. This study was to investigate if some of IgM-ICMGN were associated with autoimmune disorders induced by hydralazine. DESIGN: Seven IgM-dominant ICMGN cases were identified over 8 years...
April 29, 2024: Ultrastructural Pathology
#4
JOURNAL ARTICLE
Shubham Shukla, Aravind Sekar, Sachin Naik, Manish Rathi, Aman Sharma, Ritambhra Nada, Harbir S Kohli, Raja Ramachandran
INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) rarely coexist with systemic thrombotic microangiopathy (TMA).The TMA can be in the form of either hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). This review explores the clinical characteristics, histopathological findings, treatment options, and outcomes in patients presenting as AAV with coexisting HUS/TTP. METHODS: We conducted a search on the PubMed database and additional searches from January 1998 to September 2022 using the following terms: "ANCA", "Antineutrophil cytoplasmic antibody", "thrombotic thrombocytopenic purpura", "TTP", "thrombotic microangiopathy", "haemolytic uremic syndrome", and "HUS"...
2024: Indian Journal of Nephrology
#5
Satish Chaudhary, Reetesh Sharma, Saumya Gupta, Sita Paikra, Mohit Gupta, Bal K Upadhyay, Alok Sharma, Rajiv K Sethia
Posttransplant thrombotic microangiopathy (PT-TMA) can be caused by calcineurin inhibitors (CNIs), ischemic injury, infections, or antibody-mediated rejection (ABMR). Delayed recognition can result in allograft loss. We describe the first reported case of successful reversal of refractory PT-TMA with eculizumab in India. It highlights the importance of prompt diagnosis and benefit from an early initiation of eculizumab therapy in refractory cases.
2024: Indian Journal of Nephrology
#6
JOURNAL ARTICLE
Bahareh Gheiasi, Fakhredin Taghinezhad, Darshik Kumar Patel, Ebrahim Salimi, Mashallah Babashahi, Aliashraf Mozafari
Background : COVID-19 usually complicates respiratory failure; microvascular, macrovascular, and renal complications are common. Both micro and macrovascular complications are associated with multi-organ dysfunction and in-hospital mortality. Thrombotic microangiopathy (TMA) causes microvascular thromboses associated with organ failure, including acute kidney injury (AKI). Materials and Methods : This Retrospective Cohort study included 100 COVID-19 patients with thrombocytopenia, followed up in a university hospital's intensive care unit (ICU)...
January 1, 2024: International Journal of Hematology-oncology and Stem Cell Research
#7
JOURNAL ARTICLE
Friedrich Jung, Philippe Connes
In severe cases, SARS-CoV-2 infection leads to severe respiratory failure. Although angiotensin-converting enzyme 2 (ACE2) receptors are not expressed in red blood cells, SARS-CoV-2 can interact with red blood cells (RBCs) via several receptors or auxiliary membrane proteins. Recent data show that viral infection causes significant damage to the RBCs, altering their morphology, deformability, and aggregability. Loss of RBC deformability and/or increased aggregability favors the development of thrombotic processes in the microcirculation, as has been described to occur in COVID-19 patients...
April 1, 2024: Life
#8
JOURNAL ARTICLE
Kosuke Sonoda, Masamichi Komatsu, Yoko Ozawa, Hiroshi Yamamoto, Yuji Kamijo
TAFRO syndrome is a rare systemic inflammatory disorder with a fatal course. Nevertheless, a definitive treatment strategy has not yet been established. Anti-inflammatory therapies, including glucocorticoid treatment and immunosuppressants, have not been satisfactory. Therefore, new treatment options are needed for patients with TAFRO syndrome. The effectiveness of therapeutic plasma exchange (TPE) has mainly been reported in several case reports. In this case series study, we investigated the effect of TPE on TAFRO syndrome...
April 11, 2024: Biomedicines
#9
JOURNAL ARTICLE
Freya Bleathman, Joshua Y Kausman, Laine M Hosking, Thomas A Forbes
BACKGROUND: Atypical haemolytic uraemic syndrome (aHUS) is a rare thrombotic microangiopathy resulting from dysregulation of the alternative complement pathway, leading to multi-organ dysfunction and chronic kidney disease. Eculizumab is an anti-C5 monoclonal antibody therapy that has significantly improved aHUS disease control and patient outcomes, however it requires fortnightly intravenous dosing. This often necessitates long term central access and a high hospital attendance burden...
April 25, 2024: Journal of Paediatrics and Child Health
#10
JOURNAL ARTICLE
Adrien Joseph, Stéphanie Harel, Laurent Mesnard, Cédric Rafat, Silène Knapp, Anne Rumpler, Carole Philipponnet, Christophe Barba, Jean-Michel Rebibou, David Buob, Alexandre Hertig, Jacques Vargaftig, Jean-Michel Halimi, Bertrand Arnulf, Anne-Sophie Bretaud, Bérangère Joly, Steven Grangé, Paul Coppo
BACKGROUND AND HYPOTHESIS: Carfilzomib, a new proteasome inhibitor indicated for patients with relapsed/refractory myeloma, has been associated with cases of thrombotic microangiopathy (CFZ-TMA). The role of variants in the complement alternative pathway and therapeutic potential of complement blockade with eculizumab remain to be determined. METHODS: We report 37 cases of CFZ-TMA recorded in the French reference center for TMA with their clinical characteristics, genetic analysis and outcome according to treatments...
April 24, 2024: Nephrology, Dialysis, Transplantation
#11
JOURNAL ARTICLE
Kanako Watanabe-Kusunoki, Chenyu Li, Tâmisa Seeko Bandeira Honda, Danyang Zhao, Yoshihiro Kusunoki, John Ku, Hao Long, Martin Klaus, Chao Han, Attila Braun, Elmina Mammadova-Bach, Andreas Linkermann, Kristof Van Avondt, Mathis Richter, Oliver Soehnlein, Monika I Linder, Christoph Klein, Stefanie Steiger, Hans-Joachim Anders
Thrombotic microangiopathy (TMA) is characterized by immunothrombosis and life-threatening organ failure, but the precise underlying mechanism driving its pathogenesis remains elusive. In this study, we hypothesized that gasdermin D (GSDMD), a pore-forming protein serving as the final downstream effector of pyroptosis/interleukin (IL)-1pathway, contributes to TMA and its consequences by amplifying neutrophil maturation and subsequent necrosis. Using a murine model of focal crystalline TMA, we found that Gsdmd-deficiency ameliorated immunothrombosis, acute tissue injury and failure...
April 24, 2024: Blood
#12
Tatsunori Nagamura, Soichiro Seno, Yasumasa Sekine, Tetsuro Kiyozumi
No abstract text is available yet for this article.
2024: Acute Medicine & Surgery
#13
Soichiro Kageyama, Takeki Ohashi, Akinori Kojima
Pulmonary tumor thrombotic microangiopathy (PTTM) is a very rare condition that can lead to acute severe pulmonary hypertension and circulatory failure. It is caused by tumor cell microvascular obstruction and is usually difficult to diagnose; in fact, it is often diagnosed after death. We report the case of a patient who experienced a sudden cardiac arrest and developed severe pulmonary hypertension two days after receiving the coronavirus disease (COVID-19) vaccine. The patient was initially diagnosed with vaccine-associated myocarditis, and venoarterial extracorporeal membrane oxygenation (VA-ECMO) implantation with median sternotomy was performed...
March 2024: Curēus
#14
REVIEW
Quentin Moyon, Alexis Mathian, Matthias Papo, Alain Combes, Zahir Amoura, Marc Pineton de Chambrun
PURPOSE OF THE REVIEW: Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder that can escalate into a 'thrombotic storm' called the catastrophic antiphospholipid syndrome (CAPS), frequently requiring ICU admission for multiple organ failure. This review aims to offer insight and recent evidence on critically-ill APS patients. RECENT FINDINGS: The CAPS classification criteria define this condition as the involvement of at least three organs/systems/tissues within less than a week, caused by small vessel thrombosis, in patients with elevated antiphospholipid antibodies levels...
April 23, 2024: Current Rheumatology Reports
#15
JOURNAL ARTICLE
Mouhamed Yazan Abou-Ismail, Chong Zhang, Angela P Presson, Shruti Chaturvedi, Ana G Antun, Andrew M Farland, Ryan Woods, Ara Metjian, Yara A Park, Gustaaf de Ridder, Briana Gibson, Raj S Kasthuri, Darla K Liles, Frank Akwaa, Todd Clover, Lisa Baumann Kreuziger, Meera Sridharan, Ronald S Go, Keith R McCrae, Harsh Vardhan Upreti, Radhika Gangaraju, Nicole K Kocher, X Long Zheng, Jay S Raval, Camila Masias, Spero R Cataland, Andrew D Johnson, Elizabeth Davis, Michael D Evans, Marshall Mazepa, Ming Y Lim
BACKGROUND: Mortality due to immune-mediated thrombotic thrombocytopenic purpura (iTTP) remains significant. Predicting mortality risk may potentially help individualize treatment. The French Thrombotic Microangiopathy (TMA) Reference Score has not been externally validated in the United States. Recent advances in machine learning technology can help analyze large numbers of variables with complex interactions for the development of prediction models. OBJECTIVES: To validate the French TMA Reference Score in the United States Thrombotic Microangiopathy (USTMA) iTTP database and subsequently develop a novel mortality prediction tool, the USTMA TTP Mortality Index...
March 2024: Research and Practice in Thrombosis and Haemostasis
#16
JOURNAL ARTICLE
Masaya Iwamuro, Daisuke Ennishi, Nobuharu Fujii, Ken-Ichi Matsuoka, Takehiro Tanaka, Toshihiro Inokuchi, Sakiko Hiraoka, Motoyuki Otsuka
BACKGROUND: Endoscopic features of intestinal transplant-associated microangiopathy (iTAM) have not been comprehensively investigated. This study aimed to examine the endoscopic characteristics of patients diagnosed with iTAM. METHODS: This retrospective analysis included 14 patients pathologically diagnosed with iTAM after stem cell transplantation for hematolymphoid neoplasms (n = 13) or thalassemia (n = 1). The sex, age at diagnosis, endoscopic features, and prognosis of each patient were assessed...
April 22, 2024: BMC Gastroenterology
#17
Lawanya Singh, Daniel Matassa, Sharon Li
There have been rare reports of patients developing nephrotic syndrome and thrombotic microangiopathy (TMA) with tyrosine kinase inhibitors (TKIs). We present the case of a patient with a history of metastatic pancreatic neuroendocrine tumor (pNET), treated with sunitinib, who rapidly developed TMA and acute kidney injury. The patient was successfully treated with cessation of sunitinib and administration of steroids. This case report contributes to the growing body of literature surrounding the rare side effects of TKIs and our experience with the management of these adverse events...
March 2024: Curēus
#18
Janmejay Ashvinkumar Kunpara, Bhavya Prakash Darji, Himanshu Arvindbhai Patel, Devang Patwari, Sujit Shaileshbhai Patel, Prakash Ishwarlal Darji
Proliferative glomerulonephritis in myelofibrosis is a very rare. Mesangial proliferation and sclerosis with changes of chronic thrombotic microangiopathy have been reported, but pauci-immune focal crescentic glomerulonephritis has not been described so far. Herein, we present a 68-year-old male who was a known case of myelofibrosis and presented with rapidly progressive glomerulonephritis and nephrotic range proteinuria. He was diagnosed as anti-neutrophil cytoplasmic antibody (ANCA)-negative focal crescentic glomerulonephritis, and he responded well to a course of intravenous methylprednisolone and cyclophosphamide...
2024: Indian Journal of Nephrology
#19
MULTICENTER STUDY
Michael D Keller, Patrick J Hanley, Yueh-Yun Chi, Paibel Aguayo-Hiraldo, Christopher C Dvorak, Michael R Verneris, Donald B Kohn, Sung-Yun Pai, Blachy J Dávila Saldaña, Benjamin Hanisch, Troy C Quigg, Roberta H Adams, Ann Dahlberg, Shanmuganathan Chandrakasan, Hasibul Hasan, Jemily Malvar, Mariah A Jensen-Wachspress, Christopher A Lazarski, Gelina Sani, John M Idso, Haili Lang, Pamela Chansky, Chase D McCann, Jay Tanna, Allistair A Abraham, Jennifer L Webb, Abeer Shibli, Amy K Keating, Prakash Satwani, Pawel Muranski, Erin Hall, Michael J Eckrich, Evan Shereck, Holly Miller, Ewelina Mamcarz, Rajni Agarwal, Satiro N De Oliveira, Mark T Vander Lugt, Christen L Ebens, Victor M Aquino, Jeffrey J Bednarski, Julia Chu, Suhag Parikh, Jennifer Whangbo, Michail Lionakis, Elias T Zambidis, Elizabeth Gourdine, Catherine M Bollard, Michael A Pulsipher
Viral infections remain a major risk in immunocompromised pediatric patients, and virus-specific T cell (VST) therapy has been successful for treatment of refractory viral infections in prior studies. We performed a phase II multicenter study (NCT03475212) for the treatment of pediatric patients with inborn errors of immunity and/or post allogeneic hematopoietic stem cell transplant with refractory viral infections using partially-HLA matched VSTs targeting cytomegalovirus, Epstein-Barr virus, or adenovirus...
April 18, 2024: Nature Communications
#20
JOURNAL ARTICLE
Soumya Pandey, Akul Shrivastava, Yanping Izak Harville, Michele Cottler-Fox, Terry O Harville
BACKGROUND: Thrombotic microangiopathies (TMA) are a group of disorders with overlapping clinical features that require urgent intervention. Treatment is based on the recognition of the TMA type, which is often challenging. The aim of this study was to identify specific HLA associations with different TMA types to aid rapid diagnosis and appropriate treatment, since the HLA assay can be completed within five hours. METHODS: All 86 consecutive patients who presented to the University of Arkansas for Medical Sciences between May 2013 and January 2021 with a presumptive diagnosis of TMA were included in this study...
February 8, 2024: Hematology, Transfusion and Cell Therapy
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