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Sulfhemoglobinemia
Sulfhemoglobinemia
Specialty	Hematology
Symptoms	Cyanosis, urinary tract infection and chronic constipation
Complications	Hypoxemia, methemoglobinemia, and hypoxia
Duration	100-120 days (lifespan of red blood cells)
Causes	Sulfur medications such as phenacetin, metoclopramide, dapsone, phenzopyridine, and trimethoprim-sulfamethoxazole; hydrogen-sulfide-producing intestinal bacteria, such as Morganella morganii
Risk factors	Pulmonary arteriovenous malformation
Prevention	Avoidance of sulfur-containing compounds including drugs
Treatment	Blood transfusions

Sulfhemoglobinemia is a rare condition in which there is excess sulfhemoglobin (SulfHb) in the blood. The pigment is a greenish derivative of hemoglobin which cannot be converted back to normal, functional hemoglobin. It causes cyanosis even at low blood levels.

It is a rare blood condition in which the β-pyrrole ring of the hemoglobin molecule has the ability to bind irreversibly to any substance containing a sulfur atom.^[1]^[2] When hydrogen sulfide (H₂S) (or sulfide ions) and ferrous ions combine in the heme of hemoglobin, the blood is thus incapable of transporting oxygen to the tissues.

Presentation

Symptoms include a blueish or greenish coloration of the blood (cyanosis), skin, and mucous membranes, even though a blood count test may not show any abnormalities in the blood. This discoloration is caused by greater than 5 grams per cent of deoxyhemoglobin, or 1.5 grams per cent of methemoglobin, or 0.5 grams per cent of sulfhemoglobin, all serious medical abnormalities.^{[citation needed]}

Causes

Sulfhemoglobinemia is usually drug induced, with drugs associated with it including sulphonamides, such as sulfasalazine or sumatriptan. Another possible cause is occupational exposure to sulfur compounds.^{[citation needed]}

It can also be caused by phenazopyridine.^[3]

Diagnosis

Treatment

The condition generally resolves itself with erythrocyte (red blood cell) turnover, although blood transfusions can be necessary in extreme cases.^{[citation needed]}