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September 1969

Fundus in Sulfhemoglobinemia

Author Affiliations

New Haven, Conn; Winston-Salem, NC

From the departments of surgery and medicine, Yale University School of Medicine, New Haven, Conn (Dr. Walsh), and the Department of Ophthalmology, Bowman Gray School of Medicine of Wake Forest University, Winston-Salem, NC (Dr. Beehler).

Arch Intern Med. 1969;124(3):377-378. doi:10.1001/archinte.1969.00300190117020

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