A 56-yr-old man was admitted to our hospital with a complaint of weight loss (6 kg/3 months), and mild shortness of breath at room air. He denied all past history of smoking, excessive alcohol drinking, or chronic diseases. On physical examination, both sclerae were grossly normal, breathing sound was clear, and no cardiac murmur was heard. Abdominal sound was normoactive, and organomegaly was not clear. Arterial blood gas analysis showed pH 7.45, PaO2 65 mmHg, PaCO2 36 mmHg, and O2 saturation 90%. There was no evidence of cardiomegaly, mass shadow, or pulmonary edema in both lung fields on chest radiograph. Laboratory findings showed alanine aminotransferase of 52 U/L, total bilirubin of 1.53 mg/dL, alkaline phosphatase of 597 U/L, and γ-GTP of 126 U/L. Hepatitis B virus surface antigen and antibody to hepatitis C virus were all negative. To evaluate biochemically abnormal findings, abdominal ultrasonography and dynamic CT scan were performed, which showed an ill-defined, poorly enhanced 6.5×7×7 cm-sized mass with several daughter nodules in the left lobe of the liver (). Tests for tumor markers revealed alfa-fetoprotein of 6.19 ng/mL, CA 19-9 of 773.2 U/mL, and CEA of 615.5 ng/mL. Ultrasonography-guided needle biopsy for the liver mass was performed, and then, the histological findings were compatible with cholangiocarcinoma (). On chest CT scan for both of unexplained mild dyspnea and tumor staging, a low density due to filling defect in the left interlobar pulmonary artery was found without any evidence of other metastatic nodules (). Pulmonary perfusion scan showed multiple perfusion defects in the left lower lung fields (). Echocardiographic examination revealed no evidence of vegetation on the cardiac valves or intracardiac thrombus. Based on the radiological and symptomatic findings of the patient, the diagnosis of pulmonary thromboembolism was made. At the time of diagnosis, coagulation tests showed elevated levels of blood clotting factors, such as D-dimer of 5,690 ng/mL, fibrinogen of 746 mg/dL, fibrinogen degradation product (FDP) of 8.02 µg/mL, and positive IgM anticardiolipin antibody (aCL Ab) of 73 PL (normal limit: <20 PL). The prothrombin time (PT) was 10.8 sec (international neutralization ratio [INR]=0.98), activated partial prothrombin time (aPTT) was 33.2 sec, and other coagulation factors including protein C and S activities, lupus anticoagulant and antithrombin III are all within the normal range. To treat the pulmonary thromboemboli, anticoagulation therapy using low-molecular-weight-heparin (LMWH) in therapeutic dose of 10 IU/kg every 12 hr was given subcutaneously. The patient's symptom was relieved with LMWH treatment over time and the follow up CT scan at 3 weeks after the diagnosis showed an almost complete resolution of the thromboemboli (). Blood oxygenation was also increased to PaO2 of 83 mmHg and O2 saturation of 97%. Follow-up coagulation tests demonstrated the normalization of FDP, D-dimer, and IgM aCL Ab titer, but only a slight decrease in fibrinogen level (). Systemic chemotherapy for the cholangiocarcinoma was performed. The patient has been followed up without further thrombosis during the next 3 months.
A 73-year-old Japanese woman experienced light-headedness and dementia of unknown cause for 6 months. She had had diabetes for 20 years and had a history of surgical excision of breast cancer and laparoscopic cholecystectomy 10 and 20 years before, respectively. Her neurological disturbance had advanced, and she sought treatment at Kansai Medical University Kori Hospital.\nAt the first medical examination, no abnormalities were detected by brain computed tomography (CT) scan. However, 1 month after the consultation, the patient experienced general hypotonia with progressive dementia and was hospitalized. At the time of admission, her blood coagulation tests were as follows (normal range in parentheses): platelets 6.8 × 104/μl (14–34 × 104/μl), fibrinogen 74.0 mg/dl (180–355 mg/dl), D-dimer 101.6 μg/ml (0–0.9 μg/ml), prothrombin time 81.2% (75–130%) and activated partial thromboplastin time 27.9 s (23–35 s). Her serum albumin had decreased to 1.8 g/dl (3.8–5.0 mg/dl), and γ-GTP had increased to 172 U/l (8–45 U/l). The serum CA19-9 level was 376,100 U/ml (<37 U/ml), and the CA15-3 level was 124 U/ml (<27 U/ml), both of which were significantly increased. Upper abdominal CT scan revealed a large ill-defined mass in the periphery of the right liver lobe and multiple ischemic lesions in the spleen. Because of her poor condition, further examination of the liver mass was impossible. Cerebral magnetic resonance imaging showed multi-regional hypointense areas in the hemisphere of her right cortex, which were compatible with acute cerebral infarction. Cardiac ultrasound showed abnormal movements in the mitral and aortic valves.\nDespite continuous anticoagulant therapy, her consciousness deteriorated and she died 3 months after the hospitalization due to multiple organ failure. The postmortem examination was performed 8 h after her death. The main object of the autopsy was to elucidate the pathological cause of the cerebral infarction.