Updated international consensus report on the investigation and management of primary immune thrombocytopenia

Abstract

Over the last decade, there have been numerous developments and changes in treatment practices for the management of patients with immune thrombocytopenia (ITP). This article is an update of the International Consensus Report published in 2010. A critical review was performed to identify all relevant articles published between 2009 and 2018. An expert panel screened, reviewed, and graded the studies and formulated the updated consensus recommendations based on the new data. The final document provides consensus recommendations on the diagnosis and management of ITP in adults, during pregnancy, and in children, as well as quality-of-life considerations.

Conflict of interest statement

Conflict-of-interest disclosure: D.P. has received research support and honoraria from Amgen and Novartis and has acted as a consultant for UCB, MedImmune, and Ono. D.M.A. has received research funding from Novartis, Bristol-Myers Squibb, and Rigel and has acted as a consultant for Novartis, Principia, and Rigel. J.B.B. has acted as a consultant (including serving on advisory boards) for Amgen, Novartis, Dova Pharmaceuticals, UCB, argenx, Momenta, Rigel, Tranquil, Regeneron Pharmaceuticals, Octapharma, Kezar, and the PDSA. B.H.C. has received honorarium from Novartis for participating in the speaker’s forum. N.C. has received honoraria for educational speaking events and for serving on advisory boards for Novartis, Amgen, Rigel, and Principia and has acted as the chief investigator or principal investigator of clinical trials for Amgen, Novartis, Rigel, Principia, and UCB. T.G. has received honoraria from Amgen; has acted as a consultant for Amgen, Dova Pharmaceuticals, Biogen, Cellphire, Fujifilm, Rigel, Shionogi, and Principia; and has received research support from Principia. W.G. has received research grants from Novartis, Bayer, and BMS/Pfizer; has received lecture honoraria from Novartis, Amgen, and Bayer; and has acted as a consultant for Amgen, Novartis, and MSD. B.G. has served as an expert for Amgen and Novartis and has received research funds from Amgen and Roche. T.J.G.-L. has received research grants and speaker honoraria from Amgen and Novartis. J.G. has received honoraria (speaker fees, travel grants) from Amgen and Novartis and has acted as a consultant for Amgen, Novartis, Ono, Alexion, and Biotest. C.K. has acted as a consultant for Novartis and UCB; has received honoraria from Amgen (paid to PDSA); PDSA funding from Amgen, Argenx, Dova, Novartis, Momenta, Principia, Octapharma, CSL Behring, Rigel, and UCB; and is a board member of the Thrombosis & Hemostasis Societies of North America. V.M. has acted as a consultant for AbbVie and has received honoraria from Bayer, Novartis, and Amgen. M.M. has received consultancy fees/honoraria (for serving on advisory boards and/or for being a member of the speakers bureau) from Amgen, Novartis, and Rigel in the last 2 years. A.C.N. has acted as a consultant for and has received honoraria directly from Amgen, Angle, argenx, Dova Pharmaceuticals, Novartis, Ono, Rigel, and Shionogi; has received funding from Amgen, Novartis, and Rigel; and has provided paid expert testimony for argenx and Rigel. S.P. has received fees for lectures, chairing sessions, and serving on advisory boards. F.R. has been a member of the speakers bureau/advisory board for Amgen, Novartis, and argenx. M.S. has received honoraria from Novartis for serving on its advisory board and for being a member of its speakers bureau. Y.T. has acted as a consultant for Sysmex and has received honoraria from Novartis, Kyowa Kirin, and Chugai. R.S.W. has received research funding from Amgen, Apellis Pharmaceuticals, Bayer, Boehringer-Ingelheim, Biogen-Idec, Bristol-Myers Squibb, GlaxoSmithKline, Janssen, Merck, Novartis, Pfizer, Roche, and Sanofi and has acted as a consultant for Alexion, Amgen, Bayer, Boehringer-Ingelheim, Kyowa Kirin, and Novartis. F.Z. has received research funding from Novartis, Celgene, AbbVie, Amgen, and Janssen Cilag and has received honoraria for participating on advisory board and/or for presenting talks at meetings from Novartis, Celgene, AbbVie, Amgen, Janssen Cilag, Sandoz, Takeda, and Roche. D.J.K. has received research funding from Protalex, Bristol-Myers Squibb, Rigel, Bioverativ, Agios Pharmaceuticals, Syntimmune, Principia, and Alnylam Pharmaceuticals and has acted as a consultant for Ono Pharmaceutical, Pfizer, 3SBio, Eisai, GlaxoSmithKline, Genzyme, Shire, Amgen, Shionogi, Rigel, Syntimmune, MedImmune, Novartis, Alexion, Bioverativ, Argenx, Zafgen, Fujifilm, Principia, Kyowa Kirin, Takeda, and the Platelet Disorders Support Group. M.H. declares no competing financial interests.

© 2019 by The American Society of Hematology.

Figures

Figure 1.

Overview of therapies for the treatment of adult ITP. The evidence available for medical therapies is indicated.