Sulfhemoglobinemia

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.

Sulfhemoglobinemia
SpecialtyHematology 
Complicationscyanosis, hypoxemia, methemoglobinemia and hypoxia
Causessulfur medications and hydrogen sulfide, which produces intestinal bacteria such as Morganella morganii
Risk factorsPulmonary arteriovenous malformation

It is a rare blood condition in which the hemoglobin molecule has the ability to bind irreversibly to any substance containing a sulfur atom.[1] When hydrogen sulfide (H2S) (or sulfide ions) and ferric ions combine in the blood, the blood is incapable of carrying oxygen.

Presentation

Symptoms include a blueish or greenish coloration of the blood, skin, and mucous membranes, even though a blood count test may not show any abnormalities in the blood. This discoloration is called cyanosis, and 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.

Causes

This can be caused by taking medications that contain sulfonamides under certain conditions (i.e., overdosing of sumatriptan).Sulfhemoglobinemia is usually drug induced. Drugs associated with sulfhemoglobinemia include sulphonamides such as sulfasalazine. Another possible cause is occupational exposure to sulfur compounds.

It can be caused by phenazopyridine.[2]

Treatment

The condition generally resolves itself with erythrocyte (red blood cell) turnover, although blood transfusions can be necessary in extreme cases.

Notable cases

On June 8, 2007, Canadian anesthesiologists Dr. Stephan Schwarz, Dr. Giuseppe Del Vicario, and Dr. Alana Flexman presented an unusual case in The Lancet.[3] A 42-year-old male patient was brought into Vancouver's St. Paul's Hospital after falling asleep in a kneeling position, which caused compartment syndrome and a buildup of pressure in his legs. When doctors drew the man's blood prior to performing the surgery to relieve the pressure from the man's legs, they noted his blood was green. A sample of the blood was immediately sent to a lab. In this case, sulfhemoglobinemia was possibly caused by the patient taking higher-than-prescribed doses of sumatriptan.[4][5]

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. 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.
  3. Flexman AM, Del Vicario G, Schwarz SK (June 2007). "Dark green blood in the operating theatre". Lancet. 369 (9577): 1972. doi:10.1016/S0140-6736(07)60918-0. PMID 17560450. S2CID 39437785.
  4. "Patient bleeds dark green blood", BBC News, 8 June 2007
  5. "Dark Green Blood In The Operating Theatre", Medical News Today, June 8, 2007
Classification
External resources
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