Pathophysiology of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.

Kremer Hovinga, Johanna Anna; Heeb, Silvan Rolf; Skowronska, Magdalena; Schaller Tschan, Monica Maria (2018). Pathophysiology of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Journal of thrombosis and haemostasis, 16(4), pp. 618-629. Wiley-Blackwell 10.1111/jth.13956

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Thrombotic microangiopathies are rare disorders characterized by the concomitant occurrence of severe thrombocytopenia, microangiopathic hemolytic anemia, and a variable degree of ischemic end-organ damage. The latter particularly affects the brain, the heart, and the kidneys. The primary forms, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), although their clinical presentations often overlap, have distinctive pathophysiologies. TTP is the consequence of a severe ADAMTS-13 deficiency, either immune-mediated as a result of circulating autoantibodies, or caused by mutations in ADAMTS-13. HUS develops following an infection with Shiga-toxin producing bacteria, or as the result of excessive activation of the alternative pathway of the complement system because of mutations in genes encoding complement system proteins.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Unit Childrens Hospital > Forschungsgruppe Hämatologie (Erwachsene)

UniBE Contributor:

Kremer Hovinga, Johanna Anna; Heeb, Silvan Rolf; Skowronska, Magdalena and Schaller Tschan, Monica Maria

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1538-7836

Publisher:

Wiley-Blackwell

Language:

English

Submitter:

Pierrette Durand Lüthi

Date Deposited:

20 Aug 2018 15:16

Last Modified:

23 Oct 2019 20:17

Publisher DOI:

10.1111/jth.13956

PubMed ID:

29356300

Uncontrolled Keywords:

ADAMTS-13 Shiga toxin alternative complement pathway hemolytic uremic syndrome thrombotic thrombocytopenic purpura

BORIS DOI:

10.7892/boris.119320

URI:

https://boris.unibe.ch/id/eprint/119320

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