Sulfhemoglobinemia is one of the rarer hemoglobinopathies which reduces the oxygen carrying capacity of the red cell. The color of the cyanosis of the skin and mucous membranes in sulfhemoglobinemia is very subtly different from the other causes of cyanosis. However, this is not so in the fundus where a distinctive color change of the retinal vessels should suggest the diagnosis. This color is described as mauve. The following patient presented with this fundus change and is the reason for this report.
Sulfhemoglobinemia results from the binding of sulfur to hemoglobin. The exact chemical arrangement is not known but there appears to be a change in the binding of globin to heme and the introduction of one atom of labile sulfur. The sulfur and hemoglobin bond is permanent but does not affect the life span of the red cell. Since the average life span of the red cell is 120
Walsh TJ, Beehler C. Fundus in Sulfhemoglobinemia. Arch Intern Med. 1969;124(3):377–378. doi:10.1001/archinte.1969.00300190117020
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