See [Rosen theombotic microangiopathy](x-devonthink-item://F3C647AF-B411-4C79-AA85-8C42F04A7DFC?page=7&istart=4887&ilength=25&search=Thrombotic%20Microangiopathy) > **clinical features** > - fever > - [[thrombocytopaenia]] > - [[microangiopathic haemolytic anaemia]] > - renal impairment > - CNS changes (eg headache, seizure, ALOC) > - nausea, abdo pain, vomiting - ADAMTS13 deficiency - cleaves von willebrand factor on suface of endothelial cells - coombs negative haemolytic anaemia - severely fragmented red cells - Hb <9 - [[thrombocytopaenia]] and purpura - plt count <20 often - *normal* fibriongen, d dimer, coags - haematuria in 5-10% - renal failure in 10-25% - Microthrombi in circulation → can present with thrombotic events eg stroke, MI. Abdo pain likely due to ischemia > approximately 40% of pts die within 2 weeks if untreated > 80-90% survival with treatment **causes** - clopidogrel - pregnancy - last trimester - post-partum period - oral contraceptive pill - familial # management - plasma exchange - plasmapheresis removes vWF and ADAMTs 13 multimers - [[FFP]] replacement - glucocorticoids / splenectomy - rituximab monoclonal ab therapy - platelet transfusion -- only in the case of *immediately life-threatening haemorrhage* - **replacement may worsen intravascular thrombosis**