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Trousseau's syndrome; Trousseau's sign of malignancy; thrombophlebitis migrans
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:
1) rare
2) 10% of patients with pancreatic cancer
Pathology:
1) excess thrombin generation
2) thrombocytopenia
3) chronic disseminated intravascular coagulation (DIC)
4) hypercoagulable state
- superficial venous thrombophlebitis
- arterial thromboembolism
5) generally associated with adenocarcinoma
a) pancreatic cancer 24%
b) lung cancer 20%
c) stomach cancer 12%
d) acute leukemia 9%
e) colorectal carcinoma 5%
f) prostate cancer ?
6) 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 [4]
Clinical manifestations:
- superficial migratory thrombophlebitis +/- arterial thromboembolism
- fever is not a feature
- renal insufficiency is not a featur
Laboratory:
1) complete blood count (CBC) shows thrombocytopenia
- anemia & leukopenia may be noted
2) PT & aPTT may be prolonged or normal, INR may be elevated
3) increased fibrin degradation products
a) elevated D-dimer
b) diminished plasma fibrinogen
- may be elevated with inflammatory process
- progressively diminishes with DIC
Management:
1) the hypercoagulable state is not relieved by anticoagulation with warfarin
2) heparin, especially LMW heparin (Levonox), may be more appropriate in these patients
- heparin blocks selectin recognition of ligands
Related
disseminated intravascular coagulation (DIC)
Trousseau's sign
General
hypercoagulability associated with malignancy
syndrome
References
- Stedman's Medical Dictionary 26th ed, Williams &
Wilkins, Baltimore, 1995
- Schiller G, in: UCLA Intensive Course in Geriatric Medicine &
Board Review, Marina Del Ray, CA, Sept 12-15, 2001
- UpToDate 14.1
http://www.utdol.com
- McCarthy N
Trousseau's sign
Nature Reviews Cancer. 2005. May 1
https://www.nature.com/articles/nrc1624
- Boccaccio C et al.
The MET oncogene drives a genetic programme linking cancer to haemostasis.
Nature 2005. 434:396-400
https://www.nature.com/articles/nature03357