Nihon Toseki Igakkai Zasshi
Online ISSN : 1883-082X
Print ISSN : 1340-3451
ISSN-L : 1340-3451
Multiorgan failure and ischemic cardiomyopathy with microthrombosis–induced plasmapheresis–refractory thrombotic thrombocytopenic purpura revealed by autopsy
Ryosuke IshikawaTomoki YoshiokaJunji HiragaYukina MizunoAyae SaitoTakahiro HoriHisashi KurataShin NagaiYoriko YamashitaMichihiko Narita
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2022 Volume 55 Issue 6 Pages 387-392

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Abstract

We report an autopsy case of fulminant multiorgan failure induced by thrombotic thrombocytopenic purpura (TTP) in a 78‒year‒old male complaining of fever and fatigue. When he was referred to our hospital in August 201X, blood tests revealed thrombocytopenia, hemolysis, and severe renal dysfunction, and a disturbance of consciousness was observed, leading to a suspicion of TTP. We immediately started plasma exchange, methylprednisolone pulse therapy, and hemodialysis, but he went into cardiopulmonary arrest that night and spontaneous resuscitation, intra‒aortic balloon pumping, and extracorporeal membrane oxygenation (ECMO) were employed to save his life. On each day of the patientʼs hospitalization, plasma exchange and hemodialysis were performed, and steroid treatment was administered, but they were ineffective, and the patient died on day 7. Later, his ADAMTS13 activity was found to be below the limit of detection, and he was diagnosed with TTP. An autopsy showed ischemic multiorgan failure due to multiple microthrombi, and the cause of death was diagnosed as ischemic cardiomyopathy. In cases like this one, in which the patient does not respond to standard treatment and shows a rapid course of thrombotic multiorgan failure, another treatment strategy, such as the use of caplacizumab, may be needed.

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© 2022 The Japanese Society for Dialysis Therapy
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