Skip to main content
PMC home page
The Western Journal of Medicine logoLink to The Western Journal of Medicine
. 1993 Apr;158(4):364–371.

Trousseau's syndrome.

N Callander 1, S I Rapaport 1
PMCID: PMC1022062  PMID: 8317122

Abstract

We report 4 cases of Trousseau's syndrome, in which spontaneous recurrent or migratory venous thromboses, arterial emboli caused by nonbacterial thrombotic endocarditis, or both, develop in a patient with a recognized or occult malignant tumor. The clinical course of 3 of the patients emphasizes a key point: The occurrence for no known reason of thromboses preventable by anticoagulation therapy with heparin but not with warfarin sodium should alert a physician to focus diagnostic efforts on uncovering an underlying malignant lesion. Thromboses may occur months to years before the tumor is discovered, and a thorough negative initial examination does not obviate the need for a continuing search. Patients with Trousseau's syndrome have persistent low-grade intravascular coagulation, and therapy with heparin should be continued indefinitely. Stopping heparin therapy for even a day may permit a new thrombosis to develop. Immunostaining a biopsy specimen from 1 patient provided evidence that 2 properties of a neoplastic lesion are required for the syndrome to develop: The malignant cells express surface membrane tissue factor, and structural features of the tumor permit the malignant cells or vesicles it sheds to be exposed to circulating blood.

Full text

PDF
364

Images in this article

366Image
on p.366

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Abildgaard U., Lindahl A. K., Sandset P. M. Heparin requires both antithrombin and extrinsic pathway inhibitor for its anticoagulant effect in human blood. Haemostasis. 1991;21(4):254–257. doi: 10.1159/000216234. [DOI] [PubMed] [Google Scholar]
  2. Bell W. R., Starksen N. F., Tong S., Porterfield J. K. Trousseau's syndrome. Devastating coagulopathy in the absence of heparin. Am J Med. 1985 Oct;79(4):423–430. doi: 10.1016/0002-9343(85)90028-2. [DOI] [PubMed] [Google Scholar]
  3. Bryan C. S. Nonbacterial thrombotic endocarditis with malignant tumors. Am J Med. 1969 May;46(5):787–793. doi: 10.1016/0002-9343(69)90029-1. [DOI] [PubMed] [Google Scholar]
  4. Byrd R. B., Divertie M. B., Spittell J. A., Jr Bronchogenic carcinoma and thromboembolic disease. JAMA. 1967 Dec 11;202(11):1019–1022. [PubMed] [Google Scholar]
  5. Callander N. S., Varki N., Rao L. V. Immunohistochemical identification of tissue factor in solid tumors. Cancer. 1992 Sep 1;70(5):1194–1201. doi: 10.1002/1097-0142(19920901)70:5<1194::aid-cncr2820700528>3.0.co;2-e. [DOI] [PubMed] [Google Scholar]
  6. Dasmahapatra K. S., Cheung N. K., Spillert C., Lazaro E. An assessment of monocyte procoagulant activity in patients with solid tumors. J Surg Res. 1987 Aug;43(2):158–163. doi: 10.1016/0022-4804(87)90159-4. [DOI] [PubMed] [Google Scholar]
  7. Dvorak H. F., Quay S. C., Orenstein N. S., Dvorak A. M., Hahn P., Bitzer A. M., Carvalho A. C. Tumor shedding and coagulation. Science. 1981 May 22;212(4497):923–924. doi: 10.1126/science.7195067. [DOI] [PubMed] [Google Scholar]
  8. Edwards R. L., Rickles F. R., Cronlund M. Abnormalities of blood coagulation in patients with cancer. Mononuclear cell tissue factor generation. J Lab Clin Med. 1981 Dec;98(6):917–928. [PubMed] [Google Scholar]
  9. FISHER M. M., WILENSKY N. D. Recurrent thrombophlebitis in obscure malignant tumor of the lung; report of four cases. J Am Med Assoc. 1951 Nov 24;147(13):1213–1216. doi: 10.1001/jama.1951.03670300027005. [DOI] [PubMed] [Google Scholar]
  10. FRICK P. G. Acute hemorrhagic syndrome with hypofibrinogenemia in metastatic cancer. Acta Haematol. 1956 Jul;16(1):11–29. doi: 10.1159/000205113. [DOI] [PubMed] [Google Scholar]
  11. Falanga A., Gordon S. G. Isolation and characterization of cancer procoagulant: a cysteine proteinase from malignant tissue. Biochemistry. 1985 Sep 24;24(20):5558–5567. doi: 10.1021/bi00341a041. [DOI] [PubMed] [Google Scholar]
  12. Gonmori H., Maekawa T., Kobayashi N., Tanaka H., Tsukada H., Takada M., Andou K. The role of tissue thromboplastin in the development of DIC accompanying neoplastic diseases. Bibl Haematol. 1983;(49):23–39. doi: 10.1159/000408444. [DOI] [PubMed] [Google Scholar]
  13. LAFLER C. J., HINERMAN D. L. A morphologic study of pancreatic carcinoma with reference to multiple thrombi. Cancer. 1961 Sep-Oct;14:944–952. doi: 10.1002/1097-0142(196109/10)14:5<944::aid-cncr2820140506>3.0.co;2-1. [DOI] [PubMed] [Google Scholar]
  14. LIEBERMAN J. S., BORRERO J., URDANETA E., WRIGHT I. S. Thrombophlebitis and cancer. JAMA. 1961 Aug 26;177:542–545. doi: 10.1001/jama.1961.03040340006002. [DOI] [PubMed] [Google Scholar]
  15. Levine M. N., Gent M., Hirsh J., Arnold A., Goodyear M. D., Hryniuk W., De Pauw S. The thrombogenic effect of anticancer drug therapy in women with stage II breast cancer. N Engl J Med. 1988 Feb 18;318(7):404–407. doi: 10.1056/NEJM198802183180703. [DOI] [PubMed] [Google Scholar]
  16. MCKAY D. G., WAHLE G. H., Jr Disseminated thrombosis in colon cancer. Cancer. 1955 Sep-Oct;8(5):970–978. doi: 10.1002/1097-0142(1955)8:5<970::aid-cncr2820080520>3.0.co;2-k. [DOI] [PubMed] [Google Scholar]
  17. McKAY D. G., MANSELL H., HERTIG A. T. Carcinoma of the body of the pancreas with fibrin thrombosis and fibrinogenopenia. Cancer. 1953 Sep;6(5):862–869. doi: 10.1002/1097-0142(195309)6:5<862::aid-cncr2820060503>3.0.co;2-2. [DOI] [PubMed] [Google Scholar]
  18. Mosesson M. W., Colman R. W., Sherry S. Chronic intravascular coagulation syndrome. N Engl J Med. 1968 Apr 11;278(15):815–821. doi: 10.1056/NEJM196804112781503. [DOI] [PubMed] [Google Scholar]
  19. NUSBACHER J. MIGRATORY VENOUS THROMBOSIS AND CANCER. N Y State J Med. 1964 Sep 1;64:2166–2173. [PubMed] [Google Scholar]
  20. Ornstein D. L., Zacharski L. R., Memoli V. A., Kisiel W., Kudryk B. J., Hunt J., Rousseau S. M., Stump D. C. Coexisting macrophage-associated fibrin formation and tumor cell urokinase in squamous cell and adenocarcinoma of the lung tissues. Cancer. 1991 Sep 1;68(5):1061–1067. doi: 10.1002/1097-0142(19910901)68:5<1061::aid-cncr2820680525>3.0.co;2-d. [DOI] [PubMed] [Google Scholar]
  21. PERLOW S., DANIELS J. L. Venous thrombosis and obscure visceral carcinoma; report of ten cases. AMA Arch Intern Med. 1956 Feb;97(2):184–188. doi: 10.1001/archinte.1956.00250200060004. [DOI] [PubMed] [Google Scholar]
  22. Pineo G. F., Regoeczi E., Hatton M. W., Brain M. C. The activation of coagulation by extracts of mucus: a possible pathway of intravascular coagulation accompanying adenocarcinomas. J Lab Clin Med. 1973 Aug;82(2):255–266. [PubMed] [Google Scholar]
  23. Rapaport S. I. The extrinsic pathway inhibitor: a regulator of tissue factor-dependent blood coagulation. Thromb Haemost. 1991 Jul 12;66(1):6–15. [PubMed] [Google Scholar]
  24. Rickles F. R., Edwards R. L. Activation of blood coagulation in cancer: Trousseau's syndrome revisited. Blood. 1983 Jul;62(1):14–31. [PubMed] [Google Scholar]
  25. Rohner R. F., Prior J. T., Sipple J. H. Mucinous malignancies, venous thrombosis and terminal endocarditis with emboli. A syndrome. Cancer. 1966 Dec;19(12):1805–1812. doi: 10.1002/1097-0142(196612)19:12<1805::aid-cncr2820191207>3.0.co;2-c. [DOI] [PubMed] [Google Scholar]
  26. Sack G. H., Jr, Levin J., Bell W. R. Trousseau's syndrome and other manifestations of chronic disseminated coagulopathy in patients with neoplasms: clinical, pathophysiologic, and therapeutic features. Medicine (Baltimore) 1977 Jan;56(1):1–37. [PubMed] [Google Scholar]
  27. Szczepański M., Bardadin K., Zawadzki J., Pypno W. Procoagulant activity of gastric, colorectal, and renal cancer is factor VII-dependent. J Cancer Res Clin Oncol. 1988;114(5):519–522. doi: 10.1007/BF00391504. [DOI] [PubMed] [Google Scholar]
  28. WOMACK W. S., CASTELLANO C. J. Migratory thrombophlebitis associated with ovarian carcinoma. Am J Obstet Gynecol. 1952 Feb;63(2):467–469. doi: 10.1016/s0002-9378(15)32851-9. [DOI] [PubMed] [Google Scholar]
  29. Wojtukiewicz M. Z., Zacharski L. R., Memoli V. A., Kisiel W., Kudryk B. J., Rousseau S. M., Stump D. C. Indirect activation of blood coagulation in colon cancer. Thromb Haemost. 1989 Dec 29;62(4):1062–1066. [PubMed] [Google Scholar]
  30. Wun T. C. Lipoprotein-associated coagulation inhibitor (LACI) is a cofactor for heparin: synergistic anticoagulant action between LACI and sulfated polysaccharides. Blood. 1992 Jan 15;79(2):430–438. [PubMed] [Google Scholar]
  31. Zacharski L. R., Schned A. R., Sorenson G. D. Occurrence of fibrin and tissue factor antigen in human small cell carcinoma of the lung. Cancer Res. 1983 Aug;43(8):3963–3968. [PubMed] [Google Scholar]

Articles from Western Journal of Medicine are provided here courtesy of BMJ Publishing Group

RESOURCES