Eric Mariotte1, Agnès Veyradier. 1. aService de Réanimation médicale, Hôpital Saint-Louis bService d'Hématologie biologique, Hôpital Lariboisière, Paris, France.
Abstract
PURPOSE OF REVIEW: Thrombotic thrombocytopenic purpura (TTP) is a rare but challenging disease for intensive care specialists. Patients with acute TTP frequently require admission to the intensive care unit because of organ dysfunctions due to the disease or because of the risk of sudden aggravation at the onset of the disease. This review aims at describing recent evolutions in the diagnosis and for the management of TTP for the use of intensive care specialists. RECENT FINDINGS: The use of A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats (ADAMTS13) activity along with clinico-biological features to define TTP by most researchers' teams has led to easier interpretation of the literature. The main issues in TTP treatment in 2015 remain the indication and timing of introduction of anti-CD20 antibody rituximab for the treatment of inaugural TTP and the preemptive use of rituximab in asymptomatic patients with decreasing ADAMTS13 activity. SUMMARY: The classification of thrombotic microangiopathies has evolved from a clinical to a pathophysiological definition. TTP is characterized by a severe ADAMTS13 deficiency that can be documented in vitro, along with anti-ADAMTS13 antibodies in most adult cases. Plasmapheresis and immunosuppressive therapy with steroids remain the standard of care for acute inaugural TTP. Anti-CD20 monoclonal antibody rituximab is safe and indicated in relapsing and/or refractory TTP. Its indication in inaugural TTP remains to be evaluated but is nevertheless recommended by experts. Novel therapies for TTP are still in preclinical phases.
PURPOSE OF REVIEW: Thrombotic thrombocytopenic purpura (TTP) is a rare but challenging disease for intensive care specialists. Patients with acute TTP frequently require admission to the intensive care unit because of organ dysfunctions due to the disease or because of the risk of sudden aggravation at the onset of the disease. This review aims at describing recent evolutions in the diagnosis and for the management of TTP for the use of intensive care specialists. RECENT FINDINGS: The use of A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats (ADAMTS13) activity along with clinico-biological features to define TTP by most researchers' teams has led to easier interpretation of the literature. The main issues in TTP treatment in 2015 remain the indication and timing of introduction of anti-CD20 antibody rituximab for the treatment of inaugural TTP and the preemptive use of rituximab in asymptomatic patients with decreasing ADAMTS13 activity. SUMMARY: The classification of thrombotic microangiopathies has evolved from a clinical to a pathophysiological definition. TTP is characterized by a severe ADAMTS13 deficiency that can be documented in vitro, along with anti-ADAMTS13 antibodies in most adult cases. Plasmapheresis and immunosuppressive therapy with steroids remain the standard of care for acute inaugural TTP. Anti-CD20 monoclonal antibody rituximab is safe and indicated in relapsing and/or refractory TTP. Its indication in inaugural TTP remains to be evaluated but is nevertheless recommended by experts. Novel therapies for TTP are still in preclinical phases.
Authors: Ralf J Ludwig; Karen Vanhoorelbeke; Frank Leypoldt; Ziya Kaya; Katja Bieber; Sandra M McLachlan; Lars Komorowski; Jie Luo; Otavio Cabral-Marques; Christoph M Hammers; Jon M Lindstrom; Peter Lamprecht; Andrea Fischer; Gabriela Riemekasten; Claudia Tersteeg; Peter Sondermann; Basil Rapoport; Klaus-Peter Wandinger; Christian Probst; Asmaa El Beidaq; Enno Schmidt; Alan Verkman; Rudolf A Manz; Falk Nimmerjahn Journal: Front Immunol Date: 2017-05-31 Impact factor: 7.561