Premium
Rituximab and intermediate‐purity plasma‐derived factor VIII concentrate ( K oate®) as adjuncts to therapeutic plasma exchange for thrombotic thrombocytopenic purpura in patients with an ADAMTS13 inhibitor
Author(s) -
Pandey Soumya,
Nakagawa Mayumi,
Rosenbaum Eric R.,
Arnaoutakis Konstantinos,
Hutchins Laura F.,
Makhoul Issam,
Milojkovic Natasha,
CottlerFox Michele
Publication year - 2015
Publication title -
journal of clinical apheresis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.697
H-Index - 46
eISSN - 1098-1101
pISSN - 0733-2459
DOI - 10.1002/jca.21348
Subject(s) - medicine , thrombotic thrombocytopenic purpura , rituximab , adamts13 , therapeutic plasma exchange , immunology , antibody , gastroenterology , platelet
Thrombotic thrombocytopenic purpura (TTP) results from a congenital or acquired deficiency of the von Willebrand factor (vWF)‐cleaving protease ADAMTS13. The disease can be fatal and hence treatment should be initiated promptly. Therapeutic plasma exchange (TPE) remains the standard treatment along with adjunct therapies including steroids and immunosuppressive drugs. Addition of rituximab to TPE has been shown to be beneficial in refractory/relapsing TTP; however, TPE results in removal of rituximab from the circulation requiring more frequent dosing of rituximab to achieve a favorable outcome. The intermediate‐purity plasma‐derived Factor VIII concentrate (FVIII) Koate® contains the highest amount of ADAMTS13 activity yet reported and has been used successfully in treating congenital TTP. Here we report our experience with addition of this FVIII concentrate to rituximab, corticosteroids and TPE in three TTP patients with an ADAMTS13 inhibitor to permit withholding TPE for 48 h after rituximab infusion. J. Clin. Apheresis 30:50–54, 2015. © 2014 Wiley Periodicals, Inc.