Medical Mystery of the Week

A 57-year-old man presents with a chief complaint of painful purple toes (see photo below) of three weeks duration. During this time he had intermittent blurry vision, intermittent chest pain, fatigue, anorexia, drenching night sweats, a weight loss of 6.8 kgs, and confusion. His WBC count was 15,510 and the CRP was 32,1 mg/L. Clotting studies revealed no evidence of hypercoagulability. Routine blood cultures were negative. The results of a MRI of the brain, transesophageal echocardiogram, and CT scan of the liver are shown below. What is your diagnosis and what test(s) would you recommend to confirm your suspicions?

emboli  MRI brain   echocardiogram  CT liver

DIAGNOSIS: Nonbacterial thrombotic endocarditis (NBTE) involving the posterior leaflet of the mitral valve. Also known as marantic endocarditis, NBTE closely resembles "culture negative" endocarditis, causing fever, chills, weight loss, and signs of systemic embolization, in this case involving the toes and brain. He also had thrombi in both saphenous veins and radiographic evidence of pulmonary embolization. The underlying cause of NBTE and hypercoagulability in this patient was a poorly differentiated adenocarcinoma of the head of the pancreas which had metastasized to the liver. 

In addition to pancreatic cancer, NBTE has been found in association with cancer of the lung, colon, and prostate, and with systemic lupus erythematosus, burns, HIV infection, tuberculosis, uremia, radiation exposure, snakebites, and trauma from pulmonary catheters. Although not present in this case, disseminated intravascular coagulation can be found in up to 71% of cases. Current guidelines suggest that patients with NBTE and thromboembolism should be treated with full-dose heparin.

Case credit: Johnson JA, Everett BM, Katz JT, Loscalzo J. Painful purple toes. N Engl J Med 2010;362:67-73






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