Wednesday 28 August 2013
Trousseau’s syndrome (TS), named after the French physician Armand Trousseau who first described it in 1865, refers to recurrent or migratory spontaneous venous thrombosis, arterial embolism due to non-bacterial thrombotic endocarditis, or both, in a patient with known or occult malignancy which is usually difficult to diagnose and may even remain elusive until it is disclosed in an autopsy.
Thrombosis can occur from months to years before cancer is known, and a negative thorough initial work-up does not forgo the need for continued evaluation that will ultimately allow an earlier diagnosis.
Cryptogenic thrombosis prevented by heparin but not oral anticoagulants should prompt doctors to investigate the possibility of underlying malignancy.
Patients with TS show persistent low-grade intravascular coagulation, thus accounting for the need to treat them with full large dose low molecular weight heparin on a lifelong basis.
These patients show thrombotic diathesis that can be devastating when left untreated, and the most severe cases may lead to limb amputation in just a few hours, as a result of severe disseminated intravascular coagulation (DIC) that can happen before an actual thrombosis ensues.
Particular forms of this syndrome are phlegmasia alba dolens and phlegmasia cerulea dolens, and a variant of classic TS has been identified, combining multiple arterial and venous thrombi with DIC prone to bleeding.
Malignant neoplasms are pro-thrombotic, and anomalies are possible in each point of Virchow’s triad – blood flow (stasis), components (hypercoagulability) or vessel wall (endothelial injury). These surely are synergistic forces behind this, and many other factors such as concomitant diseases, medications and decreased motility have a role as contributing factors.
TS involves marked changes in the clotting cascade, brought about by the production and release of procoagulant substances from tumour cells.
Although it may be associated with any kind of neoplasm, TS is most often related to pancreatic, lung, prostate, gastric, colorectal, ovarian and breast cancer.
Etiology - Associations
- asymptomatic pancreatic adenocarcinoma