Benign Polycythemia: Gaisböck's Syndrome

In apparently healthy populations individuals occasionally are encountered who have an unusually plethoric appearance. Andral¹ in 1843 observed that plethora is more frequently constitutional than acquired. He noted that plethoric people may have an increased quantity of "globules" in the blood and that some of these persons have peculiar symptoms including vertigo and dizziness. But not all plethoric persons have elevated concentrations of hemoglobin. The "stout, hearty, full-blooded man with rubicund face" was mentioned by Osier² who pointed out that this appearance may persist even in the presence of anemia. Gaisböck³ in 1905 observed that some patients with hypertension and vascular disease appear plethoric or apoplectic. He described 18 patients in whom hypertension was associated with an elevated red cell count but without splenomegaly. Similar cases were reported by Staehelin ⁴ and by Lucas,⁵ who stated that hypertension was much more common in this group than in polycythemia vera. Allbutt