Journal article

Rare cause of pulmonary hypertension - pulmonary tumour thrombotic microangiopathy.

Joseph O'Brien, Nicholas Jones, Mark Horrigan, Ahmed M Al-Kaisey

BMJ Case Rep | Published : 2019

Abstract

Pulmonary tumour thrombotic microangiopathy (PTTA) is a rare but lethal cause of pulmonary hypertension (PHT). Its underlying mechanism is believed to be fibrocellular intimal proliferation and microthrombosis. It has been reported in association with gastric adenocarcinoma and breast, pancreatic and lung cancers. The diagnosis is often made on postmortem examination due to the absence of diagnostic criteria and its rare occurrence. We describe the case of a middle-aged man who presented with rapidly progressive PHT. He deteriorated into multiorgan failure despite aggressive medical therapy and died 4 weeks after his initial presentation. A postmortem examination confirmed the diagnosis of P..

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