Myriam Soucemarianadin1,2, Ygal Benhamou1,3,4, Yahsou Delmas1,5, Claire Pichereau6,7, Eric Maury1,6,7, Frédéric Pène1,8,9, Jean-Michel Halimi1,10,11, Claire Presne1,12, Jean-Marc Thouret2, Agnès Veyradier1,13,14, Paul Coppo1,6,15,16. 1. Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France. 2. Service de Réanimation, Centre Hospitalier Métropole Savoie, Chambéry, France. 3. Inserm U1096, Rouen, France. 4. Service de Médecine Interne, CHU Charles Nicolle, Rouen, France. 5. Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire, Bordeaux, France. 6. Sorbonne Université, UPMC Univ Paris 06, Paris, France. 7. Service de Réanimation Médicale, CHU Saint-Antoine, Paris, France. 8. Service de Réanimation Polyvalente, Hôpital Cochin, Paris, France. 9. Université Paris 5, Paris, France. 10. Service de néphrologie-immunologie clinique, hôpital Bretonneau, Tours, France. 11. EA 4245, université François-Rabelais 2, boulevard Tonnellé, Tours, France. 12. Service de Néphrologie - Médecine Interne, Hôpital Sud, Amiens, France. 13. Service d'Hématologie Biologique, Hôpital Lariboisière, Paris, France. 14. Univ. Paris Diderot, Sorbonne Paris Cité, Paris, France. 15. Inserm U1009, Institut Gustave Roussy, Villejuif, France. 16. Service d'Hématologie, Hôpital Saint-Antoine, Paris, France.
Abstract
BACKGROUND: Daily therapeutic plasma exchange (TPE) and rituximab improved thrombotic thrombocytopenic purpura (TTP) prognosis. In the more severe cases, salvage therapies including twice-daily TPE and/or cyclophosphamide may be proposed and require evaluation. METHODS: TTP was defined as a thrombotic microangiopathy (TMA) with severe (<10%) acquired ADAMTS13 deficiency. Among patients included in the French Reference Center for TMA registry, we considered those with a severe disease (i.e., unresponsive to daily TPE and rituximab) who received twice-daily TPE. RESULTS: Nineteen of 289 (6.6%) patients with TTP were treated by twice-daily TPE between 2008 and 2014. Twice-daily TPE was associated with rituximab in 16 cases. The median duration of twice-daily TPE treatment was 3 d (2-22 d). In 6 patients (31.6%), additional treatments (mainly pulses of cyclophosphamide) were performed because of a persistently refractory disease (4 cases) or an exacerbation (2 cases), despite twice-daily TPE. Only one patient (5.3%) died. The other 18 achieved a durable complete remission 25.5 d (13-68 d) after the first TPE. The median follow-up was 14.4 months (7 d-45 months). CONCLUSIONS: Twice-daily TPE may be an efficient strategy in the more severe TTP patients with a short-term life-threatening disease that could overcome their poor prognosis.
BACKGROUND: Daily therapeutic plasma exchange (TPE) and rituximab improved thrombotic thrombocytopenic purpura (TTP) prognosis. In the more severe cases, salvage therapies including twice-daily TPE and/or cyclophosphamide may be proposed and require evaluation. METHODS: TTP was defined as a thrombotic microangiopathy (TMA) with severe (<10%) acquired ADAMTS13 deficiency. Among patients included in the French Reference Center for TMA registry, we considered those with a severe disease (i.e., unresponsive to daily TPE and rituximab) who received twice-daily TPE. RESULTS: Nineteen of 289 (6.6%) patients with TTP were treated by twice-daily TPE between 2008 and 2014. Twice-daily TPE was associated with rituximab in 16 cases. The median duration of twice-daily TPE treatment was 3 d (2-22 d). In 6 patients (31.6%), additional treatments (mainly pulses of cyclophosphamide) were performed because of a persistently refractory disease (4 cases) or an exacerbation (2 cases), despite twice-daily TPE. Only one patient (5.3%) died. The other 18 achieved a durable complete remission 25.5 d (13-68 d) after the first TPE. The median follow-up was 14.4 months (7 d-45 months). CONCLUSIONS: Twice-daily TPE may be an efficient strategy in the more severe TTP patients with a short-term life-threatening disease that could overcome their poor prognosis.
Authors: Elie Azoulay; Philippe R Bauer; Eric Mariotte; Lene Russell; Paul Knoebl; Ignacio Martin-Loeches; Frédéric Pène; Kathryn Puxty; Pedro Povoa; Andreas Barratt-Due; Jose Garnacho-Montero; Julia Wendon; Laveena Munshi; Dominique Benoit; Michael von Bergwelt-Baildon; Marco Maggiorini; Paul Coppo; Spero Cataland; Agnès Veyradier; Andry Van de Louw Journal: Intensive Care Med Date: 2019-10-07 Impact factor: 17.440