thrombotic thrombocytopenic purpura
(vWF) metalloprotease
(=ADAMS13)
Inhibition or deficiency of ADAMTS13 (a
vWF metalloprotease)→
☆↓clevage of von Willebrand factor→
↑large uncleaved vWF multimers →
↑platelet adhesion and aggregation (microthrombi formation)
plasmaphepesis, steroids, rituximab
(warm AIHA→ splenectomy, steroids, rituximab)
hematology
UWorld
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