presentazione standard di powerpoint - islh · at autopsy: platelet-rich thrombi in arterioles and...
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Thrombotic microangiopathies
Raimondo De Cristofaro
Servizio Malattie Emorragiche e Trombotiche
Fondazione Policlinico universitario “A. Gemelli”
Polo di Oncologia - Ematologia
Istituto di Medicina Interna e Geriatria
Università Cattolica S. Cuore - Roma
Financial disclosure
Nothing to declare
*
* (Hb<12.5 gr/dl, ≥3 schistocytes/100x field, Coombs negative)
VWF, Proteolysis, and Platelet Adhesion
Blood Flow
Adhesion, Rolling, Activation, Recruitment
Normal Multimers
Protease
No Protease Thrombus“Ultra-Large”
Multimers
(TTP?)
ADAMTS-13
ADAMTS-13
Scully, SSC ISTH, Liverpool 2012
Scully, SSC ISTH, Liverpool 2012
ADAMTS13 level and anti-ADAMTS13 Abs titer ?
Useful as exclusion criterion and for prognosis
TTP diagnosis when ADAMTS13<5%
The relevance of a complete pathological
anamnesis for an early diagnosis of typical HUS !
Noris, M. et al.8
CLINICAL APPLICATIONS OF
ADAMTS-13 ASSAY
Stage
Presentation
Remission
ADAMTS-13Deficiency (<5%)
Yes
No
Yes
Implication
TTP
Other TMA forms?
Risk of relapse
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