Venous thrombo-embolism during immune-mediated thrombotic thrombocytopenic purpura is prevalent in patients with a prolonged treatment with therapeutic plasma exchange

• Published in: La revue de medecine interne Vol. 41; no. 12; pp. 809 - 813
• Main Authors: Benhamou, Y, Sauvètre, G, Grangé, S, Veyradier, A, Coppo, P
• Format: Journal Article
• Language: French
• Published: France 01.12.2020
• Subjects: Adult; Catheterization, Central Venous - adverse effects; Catheterization, Central Venous - statistics & numerical data; Female; France - epidemiology; Humans; Male; Middle Aged; Plasma Exchange - adverse effects; Plasma Exchange - statistics & numerical data; Prevalence; Purpura, Thrombotic Thrombocytopenic - complications; Purpura, Thrombotic Thrombocytopenic - epidemiology; Purpura, Thrombotic Thrombocytopenic - therapy; Registries; Risk Factors; Thrombotic Microangiopathies - epidemiology; Thrombotic Microangiopathies - etiology; Venous Thromboembolism - epidemiology; Venous Thromboembolism - etiology; France; Plasma exchange; Thrombotic thrombocytopenic purpura; thrombocytopénique; ADAMTS13; Catheter related-thrombosis; Thrombotic microangiopathies; Venous thrombosis; Purpura thrombotique; Thrombose veineuse
• ISSN: 1768-3122
• DOI: 10.1016/j.revmed.2020.06.014

Thrombotic thrombocytopenic purpura (TTP) is a devastating disease characterized by disseminated microvascular thrombosis. Despite pro-thrombotic predisposing conditions, the prevalence of macrovascular venous thrombosis event (VTE) in immune-mediated TTP (iTTP) has rarely been assessed. We reviewed data of all iTTP patients of the French reference Center for thrombotic microangiopathies registry prospectively enrolled through a 10-year period, between 2008 and 2018. Venous thrombosis included either thrombosis of central venous catheter, symptomatic deep venous thrombosis of the limbs or pulmonary embolism. Forty-eight (12.7%) VTE were diagnosed. VTE was diagnosed after a median time of 7 [IQR, 3-16] days following the first therapeutic plasma exchange (TPE) and consisted mainly in catheter-related thrombosis (73%), and to a lesser extend symptomatic deep venous thrombosis (16%), proximal pulmonary embolism (8%) and splanchnic vein thrombosis (2%). Cases with VTE (VTE+ cases), required more TPE to achieve remission (P < 0.01), and the total volume of plasma required to achieve remission was larger (P < 0.01) than for VTE- cases. There was also a trend for more rituximab use in the VTE+ cases as compared to the VTE- cases (47% vs 33%; respectively; P = 0.07). Curative anticoagulation was started in 38 cases (79%), while 6 VTE cases did not receive any antithrombotic agents, and catheter was systematically removed when catheter-related thrombosis was diagnosed. VTE+ cases had a higher number of inserted central venous catheters than VTE- cases (P < 0.05). VTE is a frequent condition occurring during iTTP management and is observed when patients require a prolonged treatment with daily TPE and multiple catheter insertions. Therapeutic strategies aimed at reducing the duration of TPE treatment in iTTP should substantially reduce this complication.