To describe an extreme presentation of the chylomicronemia syndrome resulting in multiorgan system dysfunction.

A 40-year-old African American male with no past medical history presented with multiorgan system dysfunction manifested by acute respiratory failure and acute kidney injury. He was noted to have very-high triglyceride levels (>5000 mg/dL) at admission.

An echocardiogram showed normal cardiac function. Amylase and lipase were normal. We confirmed the chylomicronemia syndrome with a triglyceride assay. The associated hyperviscosity was treated with plasmapheresis to reduce the plasma triglyceride level.

After 3 sessions of plasmapheresis, his triglyceride levels were significantly reduced, his oxygenation improved, and his acute kidney injury resolved. He was successfully extubated on day 7 of the intensive care unit stay. His diabetes and hypertriglyceridemia were newly diagnosed and drug therapy was instituted with home discharge on day 14.

Severe chylomicronemia can cause multiorgan system dysfunction related to hyperviscosity. Early institution of plasmapheresis to reduce the triglyceride-rich lipoproteins can improve tissue perfusion and prevent further organ damage.