Trousseau's syndrome; Trousseau's sign of malignancy; thrombophlebitis migrans

Introduction

superficial migratory thrombophlebitis +/- arterial thromboembolism

Etiology

• associated with visceral malignancy
  • may be occult malignancy with superficial migratory thrombophlebitis presenting prior to a diagnosis of malignancy

Epidemiology

• rare
• 10% of patients with pancreatic cancer

Pathology

• excess thrombin generation
• thrombocytopenia
• chronic disseminated intravascular coagulation (DIC)
• hypercoagulable state
  • superficial venous thrombophlebitis
  • arterial thromboembolism
• generally associated with adenocarcinoma
  • pancreatic cancer 24%
  • lung cancer 20%
  • stomach cancer 12%
  • acute leukemia 9%
  • colorectal carcinoma 5%
  • prostate cancer ?
• mucins produced by adenocarcinoma may trigger syndrome by reacting with leukocyte & platelet selectins, resulting in platelet-rich microthrombi

Genetics

• the MET oncogene upregulates plasminogen activator inhibitor type-1 & cyclooxygenase-2 resulting in migrans thrombophlebitis

Clinical manifestations

• superficial migratory thrombophlebitis +/- arterial thromboembolism
• fever is not a feature
• renal insufficiency is not a featur

Laboratory

• complete blood count (CBC) shows thrombocytopenia
  • anemia & leukopenia may be noted
• PT & aPTT may be prolonged or normal, INR may be elevated
• increased fibrin degradation products
  • elevated D-dimer
  • diminished plasma fibrinogen
    • may be elevated with inflammatory process
    • progressively diminishes with DIC

Management

• the hypercoagulable state is not relieved by anticoagulation with warfarin
• heparin, especially LMW heparin (Levonox), may be more appropriate in these patients
  • heparin blocks selectin recognition of ligands

More general terms

• hypercoagulability associated with malignancy
• syndrome

Additional terms

• disseminated intravascular coagulation (DIC)
• Trousseau's sign