Sulfhemoglobinemia
Encyclopedia
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 that occurs when a sulfur
atom
is incorporated into the hemoglobin
molecule. When hydrogen sulfide
(H2S) (or sulfide
ion
s) and ferric
ions combine in the blood, the blood
is incapable of carrying oxygen
.
s under certain conditions (i.e. overdosing of sumatriptan
).
Sulfhemoglobinemia is usually drug induced. Drugs associated with sulfhemoglobinemia include acetanilid, phenacetin
, nitrate
s, trinitrotoluene and sulfur compounds (mainly sulphonamides
, sulfasalazine). Another possible cause is occupational exposure to sulfur compounds.
It can be caused by phenazopyridine
.
s can be necessary in extreme cases.
s include a blueish or greenish discoloration of the blood, skin
, and mucous membrane
s, 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 deoxyhemaglobinemia, or 1.5 grams per cent of methemaglobinemia, or 0.5 grams per cent of sulphemaglobinemia, all serious medical abnormalities.
. A 42-year-old male patient was brought into Vancouver's St. Paul's Hospital after falling asleep in a sitting position. When doctors drew the man's blood during surgery to relieve pressure from his legs, the blood was green. A sample of the blood was immediately sent to a lab. In this case, sulfhemoglobinaemia was possibly caused by the patient taking higher-than-prescribed doses of sumatriptan.
It is also believed William Thomson, 1st Baron Kelvin
suffered from Sulfhemoglobinemia.
Blood
Blood is a specialized bodily fluid in animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells....
. The pigment is a greenish derivative of hemoglobin
Hemoglobin
Hemoglobin is the iron-containing oxygen-transport metalloprotein in the red blood cells of all vertebrates, with the exception of the fish family Channichthyidae, as well as the tissues of some invertebrates...
which cannot be converted back to normal, functional hemoglobin. It causes cyanosis
Cyanosis
Cyanosis is the appearance of a blue or purple coloration of the skin or mucous membranes due to the tissues near the skin surface being low on oxygen. The onset of cyanosis is 2.5 g/dL of deoxyhemoglobin. The bluish color is more readily apparent in those with high hemoglobin counts than it is...
even at low blood levels.
It is a rare blood condition that occurs when a sulfur
Sulfur
Sulfur or sulphur is the chemical element with atomic number 16. In the periodic table it is represented by the symbol S. It is an abundant, multivalent non-metal. Under normal conditions, sulfur atoms form cyclic octatomic molecules with chemical formula S8. Elemental sulfur is a bright yellow...
atom
Atom
The atom is a basic unit of matter that consists of a dense central nucleus surrounded by a cloud of negatively charged electrons. The atomic nucleus contains a mix of positively charged protons and electrically neutral neutrons...
is incorporated into the hemoglobin
Hemoglobin
Hemoglobin is the iron-containing oxygen-transport metalloprotein in the red blood cells of all vertebrates, with the exception of the fish family Channichthyidae, as well as the tissues of some invertebrates...
molecule. When hydrogen sulfide
Hydrogen sulfide
Hydrogen sulfide is the chemical compound with the formula . It is a colorless, very poisonous, flammable gas with the characteristic foul odor of expired eggs perceptible at concentrations as low as 0.00047 parts per million...
(H2S) (or sulfide
Sulfide
A sulfide is an anion of sulfur in its lowest oxidation state of 2-. Sulfide is also a slightly archaic term for thioethers, a common type of organosulfur compound that are well known for their bad odors.- Properties :...
ion
Ion
An ion is an atom or molecule in which the total number of electrons is not equal to the total number of protons, giving it a net positive or negative electrical charge. The name was given by physicist Michael Faraday for the substances that allow a current to pass between electrodes in a...
s) and ferric
Ferric
Ferric refers to iron-containing materials or compounds. In chemistry the term is reserved for iron with an oxidation number of +3, also denoted iron or Fe3+. On the other hand, ferrous refers to iron with oxidation number of +2, denoted iron or Fe2+...
ions combine in the blood, the blood
Blood
Blood is a specialized bodily fluid in animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells....
is incapable of carrying oxygen
Oxygen
Oxygen is the element with atomic number 8 and represented by the symbol O. Its name derives from the Greek roots ὀξύς and -γενής , because at the time of naming, it was mistakenly thought that all acids required oxygen in their composition...
.
Causes
This can be caused by taking medications that contain sulfonamideSulfonamide (medicine)
Sulfonamide or sulphonamide is the basis of several groups of drugs. The original antibacterial sulfonamides are synthetic antimicrobial agents that contain the sulfonamide group. Some sulfonamides are also devoid of antibacterial activity, e.g., the anticonvulsant sultiame...
s under certain conditions (i.e. overdosing of sumatriptan
Sumatriptan
Sumatriptan is a triptan sulfa drug containing a sulfonamide group. It is used for the treatment of migraine headaches. Sumatriptan is produced and marketed by various drug manufacturers with many different trade names such as Sumatriptan, Imitrex, Imigran, Imigran recovery.-Approval and...
).
Sulfhemoglobinemia is usually drug induced. Drugs associated with sulfhemoglobinemia include acetanilid, phenacetin
Phenacetin
Phenacetin is an analgesic, once widely used; its use has declined because of its adverse effects.-History:Phenacetin was introduced in 1887, and was used principally as an analgesic, and was one of the first synthetic fever reducers to go on the market...
, nitrate
Nitrate
The nitrate ion is a polyatomic ion with the molecular formula NO and a molecular mass of 62.0049 g/mol. It is the conjugate base of nitric acid, consisting of one central nitrogen atom surrounded by three identically-bonded oxygen atoms in a trigonal planar arrangement. The nitrate ion carries a...
s, trinitrotoluene and sulfur compounds (mainly sulphonamides
Sulfonamide (medicine)
Sulfonamide or sulphonamide is the basis of several groups of drugs. The original antibacterial sulfonamides are synthetic antimicrobial agents that contain the sulfonamide group. Some sulfonamides are also devoid of antibacterial activity, e.g., the anticonvulsant sultiame...
, sulfasalazine). Another possible cause is occupational exposure to sulfur compounds.
It can be caused by phenazopyridine
Phenazopyridine
Phenazopyridine is a chemical which, when excreted into the urine, has a local analgesic effect. It is often used to alleviate the pain, irritation, discomfort, or urgency caused by urinary tract infections, surgery, or injury to the urinary tract...
.
Prognosis and treatment
The condition generally resolves itself with erythrocyte (red blood cell) turnover, although blood transfusionBlood transfusion
Blood transfusion is the process of receiving blood products into one's circulation intravenously. Transfusions are used in a variety of medical conditions to replace lost components of the blood...
s can be necessary in extreme cases.
Presentation
SymptomSymptom
A symptom is a departure from normal function or feeling which is noticed by a patient, indicating the presence of disease or abnormality...
s include a blueish or greenish discoloration of the blood, skin
Skin
-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...
, and mucous membrane
Mucous membrane
The mucous membranes are linings of mostly endodermal origin, covered in epithelium, which are involved in absorption and secretion. They line cavities that are exposed to the external environment and internal organs...
s, 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 deoxyhemaglobinemia, or 1.5 grams per cent of methemaglobinemia, or 0.5 grams per cent of sulphemaglobinemia, all serious medical abnormalities.
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 LancetThe Lancet
The Lancet is a weekly peer-reviewed general medical journal. It is one of the world's best known, oldest, and most respected general medical journals...
. A 42-year-old male patient was brought into Vancouver's St. Paul's Hospital after falling asleep in a sitting position. When doctors drew the man's blood during surgery to relieve pressure from his legs, the blood was green. A sample of the blood was immediately sent to a lab. In this case, sulfhemoglobinaemia was possibly caused by the patient taking higher-than-prescribed doses of sumatriptan.
It is also believed William Thomson, 1st Baron Kelvin
William Thomson, 1st Baron Kelvin
William Thomson, 1st Baron Kelvin OM, GCVO, PC, PRS, PRSE, was a mathematical physicist and engineer. At the University of Glasgow he did important work in the mathematical analysis of electricity and formulation of the first and second laws of thermodynamics, and did much to unify the emerging...
suffered from Sulfhemoglobinemia.