Budd-Chiari syndrome attributed to protein C deficiency.

The protein C anticoagulant pathway is an important downregulating mechanism of the blood coagulation cascade. We report a 47-yr-old male with Budd-Chiari syndrome and occlusion of the inferior vena cava. His plasma protein C antigen level was decreased to 42%, and its activity was 34%. Other coagulable factors were normal. Protein C deficiency should be considered a possible etiological factor of the Budd-Chiari syndrome.

Retinal complications with elevated circulating plasma C5a associated with interferon-alpha therapy for chronic active hepatitis C.

Retinal hemorrhage is a complication of interferon therapy of unknown pathogenesis. We report two chronic active hepatitis C patients who developed retinal hemorrhage and/or cotton wool patches during interferon-alpha therapy 4 and 12 wk after beginning treatment. At the time of the hemorrhage, plasma-activated complement 5, a known potent intravascular aggregator of granulocytes, increased to 54 ng/ml in one patient and to 29 ng/ml in the other patient. When the hemorrhage resolved, it decreased to under 5 ng/ml. Our cases suggest that complement activation occurs in patients treated with interferon-alpha and that activation of complement 5 can lead to retinal capillary infarction and retinal hemorrhage. High levels of activated complement 5 may predict retinal artery infarction or perhaps microvascular emboli in the other organs.