scholarly journals Editorial: Thrombotic Microangiopathies, Diagnostic and Therapeutic Advances

2021 ◽  
Vol 8 ◽  
Author(s):  
Robert W. Maitta ◽  
Jay S. Raval ◽  
Hollie M. Reeves ◽  
Magali J. Fontaine
2021 ◽  
pp. 95-102
Author(s):  
Everardo Arias Torres ◽  
Yongen Chang ◽  
Sheetal Desai ◽  
Ian Chang ◽  
Jonathan E. Zuckerman ◽  
...  

Thrombotic microangiopathies (TMAs) involve multiple organ systems due to the presence of microangiopathic hemolysis. One such condition, atypical hemolytic uremic syndrome (aHUS), is a complement-mediated process that is part of a spectrum of disorders that have underlying complement dysfunction of the alternative pathway due to overactivity or decreased self-nonself discrimination by innate immunity. Complement-amplifying conditions such as pregnancy may unmask a diagnosis of aHUS. We present an important case of a pregnant 23-year-old Hispanic female who presented in mid-gestation (21 weeks) with an initial diagnosis of systemic lupus erythematosus (SLE) complicated by aHUS. She met clinical criteria for aHUS on presentation and was found to have a pathogenic CFHR1–3 homozygous deletion. She has been treated with intravenous and oral steroids, cyclophosphamide, subsequently also with plasma exchange, and finally with eculizumab with partial improvement in renal function. This case adds to the emerging literature showing that SLE and aHUS (or complement-mediated TMA) can be successfully treated with C5 blockade.


2015 ◽  
Vol 135 ◽  
pp. S30-S33 ◽  
Author(s):  
Charis von Auer ◽  
Anne-Sophie von Krogh ◽  
Johanna A. Kremer Hovinga ◽  
Bernhard Lämmle

2020 ◽  
Vol 14 (3) ◽  
pp. 213
Author(s):  
Kartik Ganesh ◽  
MAbi Abraham ◽  
JithinS Kumar ◽  
RenuS Paul ◽  
Sunita Simon ◽  
...  

2021 ◽  
Vol 29 (3) ◽  
pp. 270-273
Author(s):  
Başak Ergin ◽  
Berna Buse Kobal ◽  
Zeynep Yazıcı ◽  
Ali Hakan Kaya ◽  
Sezin Canbek ◽  
...  

Objective Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic condition characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. Thrombotic microangiopathies such as preeclampsia and HELLP syndrome are pregnancy-specific, whereas others such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome are not. In this report, we present a case at which we identified a novel mutation which led to a significant reduction of ADAMTS13 activity. Case(s) A nulliparous pregnant woman of 32-year-old presenting with epigastric pain, hypertension and low platelet count was first suspected of HELLP syndrome, but was diagnosed with congenital TTP after delivery. Conclusion HELLP syndrome co-existed with undiagnosed TTP in this case. We strive to have sufficient awareness in order to distinguish these two pathologies from each other on an antenatal basis, because the causes of the managements are entirely different.


2017 ◽  
Vol 33 (11) ◽  
pp. 2009-2025 ◽  
Author(s):  
Martin Bitzan ◽  
Jakub Zieg

2011 ◽  
Vol 26 (11) ◽  
pp. 1947-1956 ◽  
Author(s):  
Peter F. Zipfel ◽  
Gunter Wolf ◽  
Ulrike John ◽  
Karim Kentouche ◽  
Christine Skerka

Sign in / Sign up

Export Citation Format

Share Document