An autopsy case of fulminant thrombotic thrombocytopenic purpura with a rapidly fatal course that responded poorly to plasma exchange therapy

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a highly lethal disease requiring urgent intervention. The diagnostic criteria for TTP include a reduction in ADAMTS13 activity to below 10％. However, because it takes time to obtain data regarding ADAMTS13 activity, in some cases treatment should be started before such test results are available. Herein, we report a fulminant case of acquired TTP, which rapidly deteriorated, resulting in death, even though plasma exchange was performed immediately. The patient was a 46‒year‒old male. Approximately one week after the onset of flu‒like symptoms, he showed an acute deterioration of his renal function (a serum creatinine level of 3.37 mg/dL), together with thrombocytopenia and the appearance of schistocytes in his peripheral blood, which made a diagnosis of TTP highly likely. On day 2, plasma exchange therapy was initiated, but the patient responded poorly to this treatment. He then developed a disturbance of consciousness, which required steroid pulse therapy and daily plasma exchange therapy. On day 3, immediately after plasma exchange was started, the patient suddenly exhibited respiratory failure and bradycardia, leading to cardiac arrest, and he died despite cardiopulmonary resuscitation being attempted. On autopsy, multiple petechiae and microthrombi were macroscopically and microscopically observed in small blood vessels in various organs, including the heart and kidneys. Although plasma exchange and steroids are still the most effective therapies for TTP, it is important to be aware that some cases are refractory to these treatments. It is also important to always consider the possibility of TTP in cases of acute kidney injury with thrombocytopenia and to promptly start plasma exchange and steroids, even before confirming the diagnosis, especially for high‒risk patients and those that deteriorate rapidly.