Glottis oedema and interstitial pulmonary disease as a result of extramedullary haemopoiesis in polycythaemia vera rubra causing asphyctic death

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, hyp...

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Bibliographic Details
Published in:Journal of clinical pathology 2003-01, Vol.56 (1), p.79-79, Article 79
Main Authors: Tzankov, A, Krugmann, J, Mikuz, G
Format: Article
Language:English
Subjects:
asphyctic death
Asphyxia - etiology
Bone marrow
case report
Correspondence
extramedullary haemopoiesis
Fatal Outcome
glottis oedema
Hematopoiesis, Extramedullary
Humans
interstitial pulmonary disease
Laryngeal Edema - etiology
Lung Diseases, Interstitial - etiology
Male
Middle Aged
polycythaemia vera rubra
Polycythemia Vera - complications
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Summary: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 patients with PV die of an acute leukaemic transformation and about 2% develop severe myelofibrosis, with extramedullary haemopoiesis in the liver and spleen. 2 An aggressive systemic spread is a rare complication of PV.
ISSN:0021-9746
1472-4146
DOI:10.1136/jcp.56.1.79