A relatively rare chronic disease of the blood in which the red cells are increased in number. The spleen becomes enlarged, and the face is a deep red rather than truly cyanotic. Occasionally reported in childhood, it occurs mostly in middle-aged males, in which increased erythrocyte count (reaching sometimes 10.000.000 per cmm), blood volume, erythroblastic activity, and blood viscosity is associated with cyanosis and splenomegaly. Headache, gas pain, and belching are the typical presenting symptoms. Long list of other symptoms. Etiology unknown. Belongs to the group of myeloproliferative syndromes. When associated with liver cirrhosis, this disorder is known as the Mosse syndrome. It is more frequently observed in people of Jewish extraction.
- L. H. Vaquez:
Sur une forme spéciale de cyanose s’accompagnant d’hyperglobulie excessive et persistante.
Comptes rendus de la Société de biologie, Paris, 1892, 44: 384-388.
- W. Osler:
Chronic cyanosis, with polycythaemia and enlarged spleen: a new clinical entity.
American Journal of the Medical Sciences, Thorofare, N.J., 1903, 126: 187-201.
Reprinted in Medical Classics, 1939, 4: 254-275.