Diagnosis and treatment of thrombotic microangiopathy

被引：31

作者：

Thonnpson, Gemma L. ^{[1
,2
]}

Kavanagh, David ^{[1
,2
]}

机构：

[1] Newcastle Univ, Translat & Clin Res Inst, Complement Therapeut Res Grp, Newcastle Upon Tyne, Tyne & Wear, England

[2] Royal Victoria Infirm, Natl Renal Complement Therapeut Ctr, Newcastle Upon Tyne, Tyne & Wear, England

来源：

INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY | 2022年 / 44卷

关键词：

haemolytic uraemic syndrome; STEC-HUS; thrombotic microangiopathy; thrombotic thrombocytopenic purpura; HEMOLYTIC-UREMIC SYNDROME; THROMBOCYTOPENIC PURPURA; FACTOR-H; C5; INHIBITOR; COMPLEMENT; MUTATIONS; ADAMTS13; DISEASE; GENE; RAVULIZUMAB;

D O I：

10.1111/ijlh.13954

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Thrombotic microangiopathy (TMA) is characterized by thrombocytopenia, microangiopathic haemolytic anaemia and end organ damage. TMAs have varying underlying pathophysiology and can therefore present with an array of clinical presentations. Renal involvement is common as the kidney is particularly susceptible to the endothelial damage and microvascular occlusion. TMAs require rapid assessment, diagnosis, and commencement of appropriate treatment due to the high morbidity and mortality associated with them. Ground-breaking research into the pathogenesis of TMAs over the past 20 years has driven the successful development of targeted therapeutics revolutionizing patient outcomes. This review outlines the clinical presentations, pathogenesis, diagnostic tests and treatments for TMAs.

引用

页码：101 / 113

页数：13

共 95 条

[1] Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura [J].

Alwan, Ferras ;

Vendramin, Chiara ;

Vanhoorelbeke, Karen ;

Langley, Katy ;

McDonald, Vickie ;

Austin, Steve ;

Clark, Amanda ;

Lester, William ;

Gooding, Richard ;

Biss, Tina ;

Dutt, Tina ;

Cooper, Nichola ;

Chapman, Oliver ;

Cranfield, Tanya ;

Douglas, Kenny ;

Watson, H. G. ;

van Veen, J. J. ;

Sibson, Keith ;

Thomas, William ;

Manson, Lynn ;

Hill, Quentin A. ;

Benjamin, Sylvia ;

Ellis, Debra ;

Westwood, John-Paul ;

Thomas, Mari ;

Scully, Marie .

BLOOD, 2017, 130 (04) :466-471

[2] Discontinuation of Eculizumab Treatment in Atypical Hemolytic Uremic Syndrome: An Update [J].

Ardissino, Gianluigi ;

Possenti, Ilaria ;

Tel, Francesca ;

Testa, Sara ;

Salardi, Stefania ;

Ladisa, Vito .

AMERICAN JOURNAL OF KIDNEY DISEASES, 2015, 66 (01) :172-173

[3] The long-acting C5 inhibitor, ravulizumab, is effective and safe in pediatric patients with atypical hemolytic uremic syndrome naive to complement inhibitor treatment [J].

Ariceta, Gema ;

Dixon, Bradley P. ;

Kim, Seong Heon ;

Kapur, Gaurav ;

Mauch, Teri ;

Ortiz, Stephan ;

Vallee, Marc ;

Denker, Andrew E. ;

Kang, Hee Gyung ;

Greenbaum, Larry A. .

KIDNEY INTERNATIONAL, 2021, 100 (01) :225-237

[4] Long-Term Efficacy and Safety of the Long-Acting Complement C5 Inhibitor Ravulizumab for the Treatment of Atypical Hemolytic Uremic Syndrome in Adults [J].

Barbour, Thomas ;

Scully, Marie ;

Ariceta, Gema ;

Cataland, Spero ;

Garlo, Katherine ;

Heyne, Nils ;

Luque, Yosu ;

Menne, Jan ;

Miyakawa, Yoshitaka ;

Yoon, Sung-Soo ;

Kavanagh, David .

KIDNEY INTERNATIONAL REPORTS, 2021, 6 (06) :1603-1613

[5] Renal thrombotic microangiopathy in patients with cblC defect: review of an under-recognized entity [J].

Beck, Bodo B. ;

van Spronsen, FrancJan ;

Diepstra, Arjan ;

Berger, Rolf M. F. ;

Komhoff, Martin .

PEDIATRIC NEPHROLOGY, 2017, 32 (05) :733-741

[6] HIV-associated thrombotic microangiopathy in the era of highly active antiretroviral therapy: An observational study [J].

Becker, S ;

Fusco, G ;

Fusco, J ;

Balu, R ;

Gangjee, S ;

Brennan, C ;

Feinberg, J .

CLINICAL INFECTIOUS DISEASES, 2004, 39 :S267-S275

[7] Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study [J].

Bendapudi, Pavan K. ;

Hurwitz, Shelley ;

Fry, Ashley ;

Marques, Marisa B. ;

Waldo, Stephen W. ;

Li, Ang ;

Sun, Lova ;

Upadhyay, Vivek ;

Hamdan, Ayad ;

Brunner, Andrew M. ;

Gansner, John M. ;

Viswanathan, Srinivas ;

Kaufman, Richard M. ;

Uhl, Lynne ;

Stowell, Christopher P. ;

Dzik, Walter H. ;

Makar, Robert S. .

LANCET HAEMATOLOGY, 2017, 4 (04) :E157-E164

[8] Ticlopidine-associated ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in 22 persons in Japan: a report from the Southern Network on Adverse Reactions (SONAR) [J].

Bennett, Charles L. ;

Jacob, Sony ;

Dunn, Brianne L. ;

Georgantopoulos, Peter ;

Zheng, X. Long ;

Kwaan, Hau C. ;

Mckoy, June M. ;

Magwood, Jametta S. ;

Qureshi, Zaina P. ;

Bandarenko, Nicholas ;

Winters, Jeffrey L. ;

Raife, Thomas J. ;

Carey, Patricia M. ;

Sarode, Ravindra ;

Kiss, Joseph E. ;

Danielson, Constance ;

Ortel, Thomas L. ;

Clark, William F. ;

Ablin, Richard J. ;

Rock, Gail ;

Matsumoto, Masanori ;

Fujimura, Yoshihiro .

BRITISH JOURNAL OF HAEMATOLOGY, 2013, 161 (06) :896-898

[9]

Blaum BS, 2015, NAT CHEM BIOL, V11, P77, DOI [10.1038/nchembio.1696, 10.1038/NCHEMBIO.1696]

[10] Long-term outcomes and response to treatment in diacylglycerol kinase epsilon nephropathy [J].

Brocklebank, Vicky ;

Kumar, Gurinder ;

Howie, Alexander J. ;

Chandar, Jayanthi ;

Milford, David, V ;

Craze, Janet ;

Evans, Jonathan ;

Finlay, Eric ;

Freundlich, Michael ;

Gale, Daniel P. ;

Inward, Carol ;

Mraz, Martin ;

Jones, Caroline ;

Wong, William ;

Marks, Stephen D. ;

Connolly, John ;

Corner, Bronte M. ;

Smith-Jackson, Kate ;

Walsh, Patrick R. ;

Marchbank, Kevin J. ;

Harris, Claire L. ;

Wilson, Valerie ;

Wong, Edwin K. S. ;

Malina, Michal ;

Johnson, Sally ;

Sheerin, Neil S. ;

Kavanagh, David .

KIDNEY INTERNATIONAL, 2020, 97 (06) :1260-1274

← 1 2 3 4 5 6 7 8 9 10 →