A patient who presents with thrombotic thrombocytopenic purpura (TTP) can be separated based on the underlying mechanism.
Underlying mechanisms:
(1) immunologic, with antibody targeting von Willebrand factor cleaving protease (VWFCP) or its bindings sites
(2) quantitative deficiency in VWFCP, such as congenital TTP
(3) drug-induced
Finding
Immune
VWFCP Deficient
Drug-Induced
history
normal in past, recent exposure
variable family history, past history, physiologic stressor
taking drug associated with TTP
inhibitor (antibody)
present
absent
therapy
poor response to FFP; better to plasmapheresis
small amount of FFP
responds to drug discontinuation
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Specialty: Hematology Oncology, Clinical Laboratory
