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Narsoplimab for severe transplant-associated thrombotic microangiopathy
Transplantation-associated thrombotic microangiopathy (TA-TMA) is an endothelial injury syndrome linked to the overactivation of complement pathways. It manifests with microangiopathic hemolytic anemia, consum... (Source: Thrombosis Journal)
Source: Thrombosis Journal - March 13, 2023 Category: Hematology Authors: Ambreen Pandrowala, Parth Ganatra, V. P. Krishnan, Ajay Narayan Sharma, Saroj Chavan, Minnie Bodhanwala, Bharat Agarwal and Prashant Hiwarkar Tags: Case Report Source Type: research

Bruton ’s tyrosine kinase inhibition attenuates disease progression by reducing renal immune cell invasion in mice with hemolytic-uremic syndrome
In conclusion, we report here for the first time that BTK inhibition attenuates the course of disease in murine HUS. We suggest that the observed reduction of renal immune cell invasion contributes – at least in part – to this effect. Further translational studies are needed to evaluate BTK as a potential target for HUS therapy to overcome currently limited treatment options. (Source: Frontiers in Immunology)
Source: Frontiers in Immunology - February 23, 2023 Category: Allergy & Immunology Source Type: research

CFH and CFHR structural variants in atypical Hemolytic Uremic Syndrome: Prevalence, genomic characterization and impact on outcome
In conclusion, these data highlight that uncommon CFH-CFHR SVs are frequent in primary aHUS and quite rare in secondary forms. Notably, genomic rearrangements involving the CFH are associated with a poor prognosis but carriers respond to anti-complement therapy. (Source: Frontiers in Immunology)
Source: Frontiers in Immunology - January 30, 2023 Category: Allergy & Immunology Source Type: research

Furosemide ‐induced haemolytic anaemia in an extreme elderly patient
This report aims to raise awareness of this rare complication caused by commonly prescribed drugs. Despi te a negative result of a direct antiglobulin test, physicians must remain suspicious of drug-induced immune haemolytic anaemia in unclear cases of haemolysis. (Source: ESC Heart Failure)
Source: ESC Heart Failure - January 19, 2023 Category: Cardiology Authors: I ‐Wei Ho, Chin‐Chou Huang Tags: Case Report Source Type: research

The Role of Complement in Autoimmune Disease-Associated Thrombotic Microangiopathy and the Potential for Therapeutics
J Rheumatol. 2023 Jan 15:jrheum.220752. doi: 10.3899/jrheum.220752. Online ahead of print.ABSTRACTThe complement system is a tightly regulated, cascading protein network representing a key component linking the innate and humoral immune systems. However, if misdirected or dysregulated, it can be similarly damaging to host-tissue. The role of complement dysregulation on vascular endothelial cells has been well established in atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and target organ injury. Yet a great deal of complexit...
Source: Journal of Rheumatology - January 15, 2023 Category: Rheumatology Authors: Anuja Java Alfred H J Kim Source Type: research

The Role of Complement in Autoimmune Disease-Associated Thrombotic Microangiopathy and the Potential for Therapeutics
J Rheumatol. 2023 Jan 15:jrheum.220752. doi: 10.3899/jrheum.220752. Online ahead of print.ABSTRACTThe complement system is a tightly regulated, cascading protein network representing a key component linking the innate and humoral immune systems. However, if misdirected or dysregulated, it can be similarly damaging to host-tissue. The role of complement dysregulation on vascular endothelial cells has been well established in atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and target organ injury. Yet a great deal of complexit...
Source: J Rheumatol - January 15, 2023 Category: Rheumatology Authors: Anuja Java Alfred H J Kim Source Type: research