Purtscher-like retinopathy (PLR) is a rare entity related with retinal ischemia due to endothelial dysfunction and embolization. We present a case of a 17-year-old patient who presented with PLR associated with transplant-associated thrombotic microangiopathy. Visual acuity was finger counting at 1 meter in both eyes. Fundoscopy demonstrated peripapillary cotton-wool spots and hypopigmentation in the middle peripheral retina. Both eyes showed signs of macular edema with intraretinal hemorrhages. Optical coherence tomography and fundus fluorescence angiography was performed to support the diagnosis. After systemic treatments and panretinal photocoagulation, the patient's vision improved and the macular edema resolved. .
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http://dx.doi.org/10.3928/23258160-20240528-05 | DOI Listing |
Clin Chim Acta
January 2025
Department of Laboratories, Seattle Children's Hospital, Seattle, WA, United States; Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, United States. Electronic address:
Background: The complement membrane attack complex involves C5b-mediated assembly of C6-C9 polymers to form pores in cell membranes during complement activation. Inactive complexes can become soluble C5b-9 (sC5b-9) when they bind to Protein S. Elevated sC5b-9 levels are associated with increased risk of hematopoietic stem cell transplant-associated thrombotic microangiopathy (TA-TMA), a serious condition which can be improved with eculizumab therapy.
View Article and Find Full Text PDFChin Med J (Engl)
December 2024
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China.
Front Nephrol
December 2024
Renal Pathophysiology Laboratory, Hospital das Clínicas, University of São Paulo School of Medicine, São Paulo, Brazil.
In glomerulopathies, endothelial dysfunction and the presence of histological vascular lesions such as thrombotic microangiopathy, arteriolar hyalinosis, and arteriosclerosis are related to a severe clinical course and worse renal prognosis. The endothelial cell, which naturally has anti-inflammatory and anti-thrombotic regulatory mechanisms, is particularly susceptible to damage caused by various etiologies and can become dysfunctional due to direct/indirect injury or a deficiency of protective factors. In addition, endothelial regulation and protection involve participation of the complement system, factors related to angiogenesis, the renin-angiotensin system (RAS), endothelin, the glycocalyx, the coagulation cascade, interaction between these pathways, interactions between glomerular structures (the endothelium, mesangium, podocyte, and basement membrane) and interstitial structures (tubules, arterioles and small vessels).
View Article and Find Full Text PDFPediatr Transplant
February 2025
Pediatric Hematology Oncology and Stem Cell Transplant, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Background: Veno-occlusive disease (VOD) and transplant-associated thrombotic microangiopathy (TA-TMA) remain a diagnostic and therapeutic challenge for patients undergoing hematopoietic stem cell transplant (HSCT). Both VOD and TA-TMA share an underlying etiology of microvascular endothelial damage. Potential under-recognition of TA-TMA in the context of VOD leaves HSCT recipients vulnerable to additional endothelial damage, and risk of end-organ failure.
View Article and Find Full Text PDFIntensive Care Med
January 2025
Medical Intensive Care Unit, AP-HP, Saint-Louis University Hospital, Paris, France.
Purpose: Advances in therapeutic care are leading to an increase in the number of patients living with overt immunosuppression. These patients are at risk of cytomegalovirus (CMV) infection and disease that can lead to or develop during ICU admission. This manuscript aims to describe the clinical presentation, risk factors, and management of CMV infection and disease in this patient population.
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