Megathrombocytopenia Associated with Glomerulonephritis, Deafness and Aortic Cystic Medianecrosis

Autor: Aage Videbæk, O. Behnke, Mogens Sandbjerg Hansen, N. Tinggaard Pedersen
Rok vydání: 2009
Předmět:
Zdroj: Scandinavian Journal of Haematology. 21:197-205
ISSN: 0036-553X
Popis: 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 micron in diameter, elaborate maze-like membrane formations, abundance of microtubules, and the total absence of very dense granules.
Databáze: OpenAIRE