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]

References

1. Gharahbaghian, Laleh; Massoudian, Bobby; DiMassa, Giancarlo (August 2009). "Methemoglobinemia and Sulfhemoglobinemia in Two Pediatric Patients after Ingestion of Hydroxylamine Sulfate". Western Journal of Emergency Medicine. 10 (3): 197–201. ISSN 1936-900X. PMC 2729224. PMID 19718385.
2. Curry, Steven (June 6, 2007). "14". In Shannon, Michael; Borron, Stephen; Burns, Michael (eds.). Haddad and Winchester's Clinical Management of Poisoning and Drug Overdose (4 ed.). Saunders. p. 289. doi:10.1016/B978-0-7216-0693-4.50019-0. ISBN 9780721606934. Archived from the original on July 28, 2021. Retrieved July 29, 2021.
3. Gopalachar AS, Bowie VL, Bharadwaj P (June 2005). "Phenazopyridine-induced sulfhemoglobinemia". Ann Pharmacother. 39 (6): 1128–30. doi:10.1345/aph.1E557. PMID 15886294. S2CID 22812461. Archived from the original on 2013-04-19.

External links

• Cancer Web Project Online Medical Dictionary