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Glottis oedema and interstitial pulmonary disease as a result of extramedullary haemopoiesis in polycythaemia vera rubra causing asphyctic death
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- polycythaemia vera rubra
- case report
- asphyctic death
- glottis oedema
- interstitial pulmonary disease
- extramedullary haemopoiesis
Polycythaemia vera rubra (PV) is a clonal disorder of the pluripotent haemopoietic progenitor cell, with overproduction of phenotypically normal erythrocytes, granulocytes, and platelets in the absence of recognisable stimuli.1 PV usually becomes clinically apparent as a result of splenomegaly, hyperuricaemia, or aquagenic pruritus, in addition to the most important complications and leading causes of death—symptoms related to the raised blood cell mass and increased blood viscosity, such as hypertension, erythromelalgia, vertigo, visual disturbances, myocardial, mesenteric and cerebral infarctions, hepatic vein thrombosis, pulmonary thromboembolism, and heart failure.1,2 Ten per cent of …