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

Rev Med Interne. 2020 Dec;41(12):809-813. doi: 10.1016/j.revmed.2020.06.014. Epub 2020 Jul 27.
[Article in French]


Introduction: 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.

Methods: 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.

Results: 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).

Conclusion: 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.

Keywords: ADAMTS13; Catheter related-thrombosis; Plasma exchange; Purpura thrombotique; Thrombose veineuse; Thrombotic microangiopathies; Thrombotic thrombocytopenic purpura; Venous thrombosis; thrombocytopénique.

MeSH terms

  • 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