Trousseau syndrome

What is Trousseau syndrome?

Trousseau syndrome is an acquired blood clotting disorder that results in migratory thrombophlebitis (inflammation of a vein due to a blood clot) in association with an often undiagnosed malignancy.

Trousseau syndrome has many synonyms including ‘migratory superficial thrombophlebitis’, 'hypercoagulability syndrome associated with cancer', and ‘tumour-associated thromboembolism’.

Who gets Trousseau syndrome? 

Trousseau syndrome develops in the setting of either an undiagnosed malignancy or at the same time as the cancer diagnosis.

Pancreatic cancer appears to be associated with the highest risk of Trousseau syndrome, but other tumours, particularly mucin-producing adenocarcinomas (cancers that develop in the lining or inner surface of an organ) such as lung and gastric can also cause the syndrome. 

What is the cause of Trousseau syndrome?

Many mechanisms for blood clotting in Trousseau syndrome have been proposed and there is probably no single pathogenic pathway. It is likely the tumour cells release products into the bloodstream such as mucin, tissue factor, and cysteine protease, resulting in thrombin activation and fibrin deposition. Oncogene activation and tissue hypoxia may also be involved. 

What are the clinical features of Trousseau syndrome?

• Venous thrombosis - includes deep vein thrombosis, superficial thrombophlebitis
• Non-bacterial thrombotic endocarditis
• Arterial thrombosis

Superficial thrombophlebitis may involve either the arterial or venous system. Lesions appear as inflamed, reddened lines or lumps in the fat under the skin. They may occur on the trunk or extremities.

The patient may present with signs of embolism, such as blue toe syndrome.

What are the complications of Trousseau syndrome?

• Pulmonary embolus
• Cerebral infarction (stroke) due to arterial thrombosis or emboli; may involve multiple sites 

How is Trousseau syndrome diagnosed?

A patient with unexplained acute thromboembolism may be investigated with abdominal/pelvic CT scan and mammography for an occult malignancy.

What is the differential diagnosis of Trousseau syndrome? 

• Cellulitis
• Erythema nodosum
• Lymphangitis
• Vasculitis

What is the treatment for Trousseau syndrome?

• Treat the underlying malignancy
• Heparin

What is the outcome of Trousseau syndrome?

Trousseau syndrome is the second commonest cause of death in cancer patients. It can progress rapidly, resulting in death. However if the underlying cancer is cured, the thromboembolism resolves.