Trousseau's syndrome in association with cholangiocarcinoma: positive tests for coagulation factors and anticardiolipin antibody

Abstract

Thromboembolic events are reported to occur with a high frequency in the setting of malignancy. However, reports on an association between cholangiocarcinoma and pulmonary thromboembolism, thus far, are almost lacking. We present here an unusual case of a 56-yr-old patient presenting cholangiocarcinoma and unexplained pulmonary thromboembolism. The patient had been quite healthy before the diagnosis. Coagulation tests showed elevated levels of fibrinogen, fibrinogen degradation product (FDP), D-dimer, and IgM anticardiolipin antibody (aCL Ab). The thromboemboli were resolved 3 weeks after anticoagulant therapy using low molecular-weight-heparin. Then, follow-up coagulation tests showed a marked decrease to normal in aCL Ab titer as well as the normalization of FDP and D-dimer levels. In this case, we describe pulmonary thromboembolism caused by hypercoagulable state associated with cholangiocarcinoma and speculate that such a thrombotic phenomenon could be regressed by anticoagulant therapy.

Fig. 1

Abdominal dynamic CT scan show about 6.5×7×7 cm-sized and ill-defined mass with several daughter nodules in the left lobe. The huge mass with a dilatation of intrahepatic bile ducts is not enhanced on the arterial phase (A), but shows delayed enhancement on the portal phase (B), indicating cholagiocarcinoma.

Fig. 2

Photomicrograph of liver biopsy specimens. Moderately differentiated adenocarcinoma is shown in the hematoxylin-eosin stain (A; original magnification ×100). On the immunohistochemical staining by using cytokeratin 19 (CK 19), dark-brown staining patterns are observed on the epithelium of proliferating bile ducts (B; original magnification ×400).

Fig. 3

Initial chest CT scan shows (A) a filling defect with lower density in the left interlobar pulmonary artery, indicating pulmonary thromboembolism. (B) Follow-up chest CT scan after anticoagulant therapy over 3 weeks revealed the interval regression of the thromboembolism.

Fig. 4

Ventilation-perfusion scan reveals that no definite ventilatory abnormality in both lungs is shown (A), but multiple perfusion defects are found on the left lower lobe (B).

Fig. 5

Changes in FDP, D-dimer, fibrinogen, and aCL Ab levels during anticoagulant therapy. The levels of FDP, D-dimer, and aCL Ab were normalized, but fibrinogen level was slightly decreased.