Thrombotic thrombocytopenic purpura: yesterday, today, tomorrow.
Although much has been learned about the pathophysiologic process of thrombotic thrombocytopenic purpura (TTP), both diagnostically and therapeutically, since its initial description by Moschcowitz in 1924, its etiology and treatments remain, in many instances, problematic. Thrombotic thrombocytopenic purpura remains a rare entity whose etiology is usually unknown, but several drugs and infections have now been implicated in its development (i.e. Cyclosporine A, Mitomycin-C, Ticlopidine, Simvastatin, Lipitor, Plavix, FK 506, Rapamune (sirolimus), HIV). Although its treatment by plasma exchange has gained worldwide acceptance since the late 1970s, the optimal exchange media is not known, nor the volume and duration of exchange therapy, nor appropriate salvage therapy(ies). Without the benefit of randomized controlled trials, its treatment, to a large extent, remains not evidence-based but 'eminence-based', making the same mistakes with increasing confidence over an impressive number of years.
McCarthy
Leo J
LJ
Department of Pathology and Laboratory Medicine (Transfusion Medicine), Indiana University School of Medicine, Indianapolis, Indiana 46202, USA. lmmcarth@iupui.edu
Dlott
Jeffrey S
JS
Orazi
Attilio
A
Waxman
Daniel
D
Miraglia
Charles C
CC
Danielson
Constance F M
CF
D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D010956:Plasmapheresis / Q000379:methods; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*; D013156:Splenectomy
D007166:Immunosuppressive Agents