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Sulfhemoglobinemia

From Wikipedia, the free encyclopedia
Sulfhemoglobinemia
SpecialtyHematology
SymptomsCyanosis, urinary tract infection and chronic constipation
ComplicationsHypoxemia, methemoglobinemia, and hypoxia
Duration100-120 days (lifespan of red blood cells)
CausesSulfur medications such as phenacetin, metoclopramide, dapsone, phenzopyridine, and trimethoprim-sulfamethoxazole; hydrogen-sulfide-producing intestinal bacteria, such as Morganella morganii
Risk factorsPulmonary arteriovenous malformation
PreventionAvoidance of sulfur-containing compounds including drugs
TreatmentBlood 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べーた-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

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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

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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

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Treatment

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

References

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  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.{{cite book}}: CS1 maint: bot: original URL status unknown (link)
  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.
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