An Autopsy Case of Mixed Connective Tissue Disease Complicated by Thrombotic Thrombocytopenic Purpura

Abstract

We herein report a patient with mixed connective tissue disease (MCTD) who had been stable for years but suddenly developed thrombotic thrombocytopenic purpura (TTP). The patient showed a clinical pentad of signs of TTP, low activity of ADAMTS13, and positivity of anti-ADAMTS13 antibodies. She did not respond to plasma exchange or steroid therapy and died five days after admission. An autopsy revealed microthrombi in the brain, heart, kidney, adrenal glands, esophageal submucosa, and bone marrow as well as diffuse alveolar hemorrhaging. Physicians should bear in mind that TTP can occur in MCTD patients regardless of disease activity.

Educational Case: Differentiating Thrombotic Thrombocytopenic Purpura From Other Thrombotic Microangiopathies and Potential Role of the Spleen

“…Additionally, patients with HUS tend to be younger than patients with TTP, but exceptions often occur. 7 , 18 , 19 Secondary causes of HUS are more challenging to exclude. In terms of laboratory testing, measuring ADAMTS13 activity levels can be informative.…”