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Megathrombocytopenia Associated with Glomerulonephritis, Deafness and Aortic Cystic Medianecrosis
Author(s) -
Hansen Mogens Sandbjerg,
Behnke O.,
Pedersen N. Tinggaard,
Videbæk Aage
Publication year - 1978
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1978.tb00354.x
Subject(s) - glomerulonephritis , medicine , pathology , aorta , kidney
Macrothrombocytopenia associated with glomerulonephritis and deafness has only been reported in a few patients since it was first described in 1972. We present a patient with macrothrombocytopenia, glomerulonephritis, progressive uraemia and progressive sensorineural deafness associated with cystic medianecrosis of the ascending aorta and malformation of the aortic valves. The patient had consistently low platelet counts but no clinical signs of a defect haemostasis. The giant platelets were characterized by their size, averaging 11 μm in diameter, elaborate maze‐like membrane formations, abundance of microtubules, and the total absence of very dense granules.