![Blausen 0683 OsmoticFlow Hypertonic](https://upload.wikimedia.org/wikipedia/commons/1/12/Blausen_0683_OsmoticFlow_Hypertonic.png)
Hemolysis or haemolysis (),
also known by
several other names, is the rupturing (
lysis) of
red blood cell
Red blood cells (RBCs), also referred to as red cells, red blood corpuscles (in humans or other animals not having nucleus in red blood cells), haematids, erythroid cells or erythrocytes (from Greek ''erythros'' for "red" and ''kytos'' for "holl ...
s (erythrocytes) and the release of their contents (
cytoplasm
In cell biology, the cytoplasm is all of the material within a eukaryotic cell, enclosed by the cell membrane, except for the cell nucleus. The material inside the nucleus and contained within the nuclear membrane is termed the nucleoplasm. ...
) into surrounding fluid (e.g.
blood plasma
Blood plasma is a light amber-colored liquid component of blood in which blood cells are absent, but contains proteins and other constituents of whole blood in suspension. It makes up about 55% of the body's total blood volume. It is the intr ...
). Hemolysis may occur
in vivo
Studies that are ''in vivo'' (Latin for "within the living"; often not italicized in English) are those in which the effects of various biological entities are tested on whole, living organisms or cells, usually animals, including humans, and ...
or
in vitro
''In vitro'' (meaning in glass, or ''in the glass'') studies are performed with microorganisms, cells, or biological molecules outside their normal biological context. Colloquially called " test-tube experiments", these studies in biology ...
.
One cause of hemolysis is the action of
hemolysin
Hemolysins or haemolysins are lipids and proteins that cause lysis of red blood cells by disrupting the cell membrane. Although the lytic activity of some microbe-derived hemolysins on red blood cells may be of great importance for nutrient acqu ...
s, toxins that are produced by certain
pathogenic bacteria or
fungi
A fungus ( : fungi or funguses) is any member of the group of eukaryotic organisms that includes microorganisms such as yeasts and molds, as well as the more familiar mushrooms. These organisms are classified as a kingdom, separately from ...
. Another cause is intense physical exercise.
Hemolysin
Hemolysins or haemolysins are lipids and proteins that cause lysis of red blood cells by disrupting the cell membrane. Although the lytic activity of some microbe-derived hemolysins on red blood cells may be of great importance for nutrient acqu ...
s damage the red blood cell's
cytoplasmic membrane
The cell membrane (also known as the plasma membrane (PM) or cytoplasmic membrane, and historically referred to as the plasmalemma) is a biological membrane that separates and protects the interior of all cells from the outside environment (th ...
, causing lysis and eventually cell death.
Etymology
From hemo- + -lysis, from , "blood") + , "loosening").
Inside the body
Hemolysis inside the body can be caused by a large number of medical conditions, including some parasites (''e.g.'', ''
Plasmodium
''Plasmodium'' is a genus of unicellular eukaryotes that are obligate parasites of vertebrates and insects. The life cycles of ''Plasmodium'' species involve development in a blood-feeding insect host which then injects parasites into a ver ...
''), some autoimmune disorders (''e.g.'', autoimmune haemolytic anaemia, drug-induced
hemolytic anemia
Hemolytic anemia or haemolytic anaemia is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells (RBCs), either in the blood vessels (intravascular hemolysis) or elsewhere in the human body (extravascular). This most commonly ...
,
atypical hemolytic uremic syndrome
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare, life-threatening, progressive disease that frequently has a genetic component. In most cases it can be effectively controlled by interruption of the complement cascade. Particular mo ...
(aHUS)),
some genetic disorders (''e.g.'',
Sickle-cell disease
Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blo ...
or
G6PD deficiency
Glucose-6-phosphate dehydrogenase deficiency (G6PDD), which is the most common enzyme deficiency worldwide, is an inborn error of metabolism that predisposes to red blood cell breakdown. Most of the time, those who are affected have no symptoms. ...
), or blood with too low a solute concentration (
hypotonic
In chemical biology, tonicity is a measure of the effective osmotic pressure gradient; the water potential of two solutions separated by a partially-permeable cell membrane. Tonicity depends on the relative concentration of selective membrane-imp ...
to cells).
Hemolysis can lead to
hemoglobinemia
Hemoglobinemia (or haemoglobinaemia) is a medical condition in which there is an excess of hemoglobin in the blood plasma. This is an effect of intravascular hemolysis, in which hemoglobin separates from red blood cells, a form of anemia.
Hemogl ...
due to
hemoglobin
Hemoglobin (haemoglobin BrE) (from the Greek word αἷμα, ''haîma'' 'blood' + Latin ''globus'' 'ball, sphere' + ''-in'') (), abbreviated Hb or Hgb, is the iron-containing oxygen-transport metalloprotein present in red blood cells (erythrocyt ...
released into the
blood plasma
Blood plasma is a light amber-colored liquid component of blood in which blood cells are absent, but contains proteins and other constituents of whole blood in suspension. It makes up about 55% of the body's total blood volume. It is the intr ...
, which plays a significant role in the
pathogenesis
Pathogenesis is the process by which a disease or disorder develops. It can include factors which contribute not only to the onset of the disease or disorder, but also to its progression and maintenance. The word comes from Greek πάθος ''pat ...
of
sepsis
Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is follo ...
and can lead to increased risk of infection due to its inhibitory effects on the
innate immune system.
Parasitic hemolysis
Because the feeding process of the ''
Plasmodium
''Plasmodium'' is a genus of unicellular eukaryotes that are obligate parasites of vertebrates and insects. The life cycles of ''Plasmodium'' species involve development in a blood-feeding insect host which then injects parasites into a ver ...
'' parasites damages red blood cells,
malaria
Malaria is a mosquito-borne infectious disease that affects humans and other animals. Malaria causes symptoms that typically include fever, tiredness, vomiting, and headaches. In severe cases, it can cause jaundice, seizures, coma, or death. S ...
is sometimes called "parasitic hemolysis" in medical literature.
HELLP, pre-eclampsia, or eclampsia
:See
HELLP syndrome
HELLP syndrome is a complication of pregnancy; the acronym stands for hemolysis, elevated liver enzymes, and low platelet count. It usually begins during the last three months of pregnancy or shortly after childbirth. Symptoms may include feelin ...
,
Pre-eclampsia, and
Eclampsia
Eclampsia is the onset of seizures (convulsions) in a woman with pre-eclampsia. Pre-eclampsia is one of the hypertensive disorders of pregnancy that presents with three main features: new onset of high blood pressure, large amounts of protein in ...
Hemolytic disease of the newborn
Hemolytic disease of the newborn is an autoimmune disease resulting from the mother's antibodies crossing the placenta to the fetus. This most often occurs when the mother has previously been exposed to blood antigens present on the fetus but foreign to her, through either a blood transfusion or a previous pregnancy.
Hemolytic anemia
Because ''in vivo'' hemolysis destroys red blood cells, in uncontrolled, chronic or severe cases it can lead to
hemolytic anemia
Hemolytic anemia or haemolytic anaemia is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells (RBCs), either in the blood vessels (intravascular hemolysis) or elsewhere in the human body (extravascular). This most commonly ...
.
Hemolytic crisis
A hemolytic crisis, or hyperhemolytic crisis, is characterized by an accelerated rate of red blood cell destruction leading to
anemia
Anemia or anaemia (British English) is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, or a reduction in the amount of hemoglobin. When anemia comes on slowly, t ...
,
jaundice, and
reticulocytosis. Hemolytic crises are a major concern with
sickle-cell disease
Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blo ...
and
G6PD deficiency
Glucose-6-phosphate dehydrogenase deficiency (G6PDD), which is the most common enzyme deficiency worldwide, is an inborn error of metabolism that predisposes to red blood cell breakdown. Most of the time, those who are affected have no symptoms. ...
.
Toxic agent ingestion or poisoning
''
Paxillus involutus
''Paxillus involutus'', commonly known as the brown roll-rim, common roll-rim is a basidiomycete fungus that is widely distributed across the Northern Hemisphere. It has been inadvertently introduced to Australia, New Zealand, South Africa, ...
'' ingestion can cause hemolysis.
Space hemolysis
Spaceflight can cause hemolysis.
Intrinsic causes
Hemolysis may result from intrinsic defects in the red blood cell itself:
* Defects of red blood cell membrane production (as in
hereditary spherocytosis
Hereditary spherocytosis (HS) is a congenital hemolytic disorder, wherein a genetic mutation coding for a structural membrane protein phenotype leads to a spherical shaping of erythrocytic cellular morphology. As erythrocytes are sphere-shaped (s ...
and
hereditary elliptocytosis
Hereditary elliptocytosis, also known as ovalocytosis, is an inherited blood disorder in which an abnormally large number of the person's red blood cells are elliptical rather than the typical biconcave disc shape. Such morphologically distinctive ...
)
* Defects in hemoglobin production (as in
thalassemia,
sickle-cell disease
Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blo ...
and
congenital dyserythropoietic anemia)
* Defective red cell metabolism (as in
glucose-6-phosphate dehydrogenase deficiency
Glucose-6-phosphate dehydrogenase deficiency (G6PDD), which is the most common enzyme deficiency worldwide, is an inborn error of metabolism that predisposes to red blood cell breakdown. Most of the time, those who are affected have no symptoms. ...
and
pyruvate kinase deficiency
Pyruvate kinase deficiency is an inherited metabolic disorder of the enzyme pyruvate kinase which affects the survival of red blood cells. Both autosomal dominant and recessive inheritance have been observed with the disorder; classically, and ...
)
*
Paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system. This destructive process occu ...
(PNH), sometimes referred to as Marchiafava-Micheli syndrome, is a rare, acquired, potentially life-threatening disease of the blood characterized by complement-induced intravascular hemolytic anemia.
Extrinsic causes
Extrinsic hemolysis is caused by the red blood cell's environment:
* Immune-mediated causes could include transient factors as in ''
Mycoplasma pneumoniae
''Mycoplasma pneumoniae'' is a very small bacterium in the class Mollicutes. It is a human pathogen that causes the disease mycoplasma pneumonia, a form of atypical bacterial pneumonia related to cold agglutinin disease. ''M. pneumoniae'' is c ...
'' infection (
cold agglutinin disease
Cold agglutinin disease (CAD) is a rare disease, rare autoimmune disease characterized by the presence of high concentrations of circulating cold sensitive antibodies, usually IgM and autoantibodies that are also active at temperatures below , dir ...
) or permanent factors as in
autoimmune diseases like
autoimmune hemolytic anemia
Autoimmune hemolytic anemia (AIHA) occurs when antibodies directed against the person's own red blood cells (RBCs) cause them to burst (lyse), leading to an insufficient number of oxygen-carrying red blood cells in the circulation. The lifetime of ...
(itself more common in diseases such as
systemic lupus erythematosus,
rheumatoid arthritis
Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are invol ...
,
Hodgkin's lymphoma
Hodgkin lymphoma (HL) is a type of lymphoma, in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells (RS cells) are present in the patient's lymph nodes. The condition w ...
, and
chronic lymphocytic leukemia).
*
Spur cell hemolytic anemia
* Any of the causes of
hypersplenism
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulati ...
(increased activity of the spleen), such as
portal hypertension.
* Acquired hemolytic anemia is also encountered in
burn
A burn is an injury to skin, or other tissues, caused by heat, cold, electricity, chemicals, friction, or ultraviolet radiation (like sunburn). Most burns are due to heat from hot liquids (called scalding), solids, or fire. Burns occur ma ...
s and as a result of certain infections (e.g.
malaria
Malaria is a mosquito-borne infectious disease that affects humans and other animals. Malaria causes symptoms that typically include fever, tiredness, vomiting, and headaches. In severe cases, it can cause jaundice, seizures, coma, or death. S ...
).
*
Lead poisoning or poisoning by
arsine
Arsine (IUPAC name: arsane) is an inorganic compound with the formula As H3. This flammable, pyrophoric, and highly toxic pnictogen hydride gas is one of the simplest compounds of arsenic. Despite its lethality, it finds some applications in ...
or
stibine causes non-immune hemolytic anemia.
*
Runners can develop hemolytic anemia due to "
footstrike hemolysis", the destruction of red blood cells in feet at foot impact.
* Low-grade hemolytic anemia occurs in 70% of prosthetic heart valve recipients, and severe hemolytic anemia occurs in 3%.
Intravascular hemolysis
Intravascular hemolysis describes hemolysis that happens mainly inside the
vasculature
The blood circulatory system is a system of organs that includes the heart, blood vessels, and blood which is circulated throughout the entire body of a human or other vertebrate. It includes the cardiovascular system, or vascular system, tha ...
.
As a result, the contents of the red blood cell are released into the general circulation, leading to
hemoglobinemia
Hemoglobinemia (or haemoglobinaemia) is a medical condition in which there is an excess of hemoglobin in the blood plasma. This is an effect of intravascular hemolysis, in which hemoglobin separates from red blood cells, a form of anemia.
Hemogl ...
and increasing the risk of ensuing
hyperbilirubinemia
Bilirubin (BR) (Latin for "red bile") is a red-orange compound that occurs in the normal catabolic pathway that breaks down heme in vertebrates. This catabolism is a necessary process in the body's clearance of waste products that arise from the ...
.
Intravascular hemolysis may occur when red blood cells are targeted by
autoantibodies, leading to
complement
A complement is something that completes something else.
Complement may refer specifically to:
The arts
* Complement (music), an interval that, when added to another, spans an octave
** Aggregate complementation, the separation of pitch-clas ...
fixation, or by damage by parasites such as ''
Babesia
''Babesia'', also called ''Nuttallia'', is an apicomplexan parasite that infects red blood cells and is transmitted by ticks. Originally discovered by the Romanian bacteriologist Victor Babeș in 1888, over 100 species of ''Babesia'' have since ...
''.
Additionally, thrombotic microangiopathy (TMA) can result in hemolysis of red blood cells. TMA is frequently observed in
aHUS patients where clots form in the small vessels of the kidney resulting in damaged red blood cells as they attempt to pass through the restricted vessels.
Extravascular hemolysis
Extravascular hemolysis refers to hemolysis taking place in the
liver
The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...
,
spleen
The spleen is an organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes . ,
bone marrow, and
lymph nodes
A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that inclu ...
.
In this case little hemoglobin escapes into
blood plasma
Blood plasma is a light amber-colored liquid component of blood in which blood cells are absent, but contains proteins and other constituents of whole blood in suspension. It makes up about 55% of the body's total blood volume. It is the intr ...
.
The
macrophages
Macrophages (abbreviated as M φ, MΦ or MP) ( el, large eaters, from Greek ''μακρός'' (') = large, ''φαγεῖν'' (') = to eat) are a type of white blood cell of the immune system that engulfs and digests pathogens, such as cancer ce ...
of the
reticuloendothelial system
In anatomy the term "reticuloendothelial system" (abbreviated RES), often associated nowadays with the mononuclear phagocyte system (MPS), was originally launched by the beginning of the 20th century to denote a system of specialised cells that eff ...
in these organs
engulf and destroy structurally-defective red blood cells, or those with antibodies attached, and release unconjugated bilirubin into the blood plasma circulation.
Typically, the spleen destroys mildly abnormal red blood cells or those coated with
IgG-type antibodies,
while severely abnormal red blood cells or those coated with
IgM-type antibodies are destroyed in the circulation or in the liver.
If extravascular hemolysis is extensive,
hemosiderin
Hemosiderin image of a kidney viewed under a microscope. The brown areas represent hemosiderin
Hemosiderin or haemosiderin is an iron-storage complex that is composed of partially digested ferritin and lysosomes. The breakdown of heme gives rise ...
can be deposited in the spleen, bone marrow, kidney, liver, and other organs, resulting in
hemosiderosis
Hemosiderosis is a form of iron overload disorder resulting in the accumulation of hemosiderin.
Types include:
* Transfusion hemosiderosis
* Idiopathic pulmonary hemosiderosis
* Transfusional diabetes
Organs affected:
* Hemosiderin deposit ...
.
Outside the body
![Hemolysis](https://upload.wikimedia.org/wikipedia/commons/7/7f/Hemolysis.jpg)
''
In vitro
''In vitro'' (meaning in glass, or ''in the glass'') studies are performed with microorganisms, cells, or biological molecules outside their normal biological context. Colloquially called " test-tube experiments", these studies in biology ...
'' hemolysis can be caused by improper technique during collection of blood specimens, by the effects of mechanical processing of blood, or by bacterial action in cultured blood specimens.
From specimen collection
Most causes of ''in vitro'' hemolysis are related to specimen collection. Difficult collections, unsecure line connections, contamination, and incorrect needle size, as well as improper tube mixing and incorrectly filled tubes are all frequent causes of hemolysis. Excessive suction can cause the red blood cells to be smashed on their way through the
hypodermic needle
A hypodermic needle (from Greek ὑπο- (''hypo-'' = under), and δέρμα (''derma'' = skin)), one of a category of medical tools which enter the skin, called sharps, is a very thin, hollow tube with one sharp tip. It is commonly used w ...
owing to turbulence and physical forces. Such hemolysis is more likely to occur when a patient's veins are difficult to find or when they collapse when blood is removed by a syringe or a modern vacuum tube. Experience and proper technique are key for any
phlebotomist
Phlebotomy is the process of making a puncture in a vein, usually in the arm, with a cannula for the purpose of drawing blood. The procedure itself is known as a venipuncture, which is also used for intravenous therapy. A person who performs a ...
, nurse or doctor to prevent hemolysis.
''In vitro'' hemolysis during specimen collection can cause inaccurate laboratory test results by contaminating the surrounding plasma with the contents of hemolyzed red blood cells. For example, the concentration of
potassium
Potassium is the chemical element with the symbol K (from Neo-Latin ''kalium'') and atomic number19. Potassium is a silvery-white metal that is soft enough to be cut with a knife with little force. Potassium metal reacts rapidly with atmosph ...
inside red blood cells is much higher than in the plasma and so an elevated potassium level is usually found in biochemistry tests of hemolyzed blood.
After the blood collection process, ''in vitro'' hemolysis can still occur in a sample due to external factors, such as prolonged storage, incorrect storage conditions and excessive physical forces by dropping or vigorously mixing the tube.
From mechanical blood processing during surgery
In some surgical procedures (especially some heart operations) where substantial blood loss is expected, machinery is used for
intraoperative blood salvage
Intraoperative blood salvage (IOS), also known as cell salvage, is a specific type of autologous blood transfusion. Specifically IOS is a medical procedure involving recovering blood lost during surgery and re-infusing it into the patient. It is ...
. A centrifuge process takes blood from the patient, washes the red blood cells with
normal saline
Saline (also known as saline solution) is a mixture of sodium chloride (salt) and water. It has a number of uses in medicine including cleaning wounds, removal and storage of contact lenses, and help with dry eyes. By injection into a vein i ...
, and returns them to the patient's blood circulation. Hemolysis may occur if the centrifuge rotates too quickly (generally greater than 500 rpm)—essentially this is hemolysis occurring outside of the body. Increased hemolysis occurs with massive amounts of sudden blood loss, because the process of returning a patient's cells must be done at a correspondingly higher speed to prevent
hypotension
Hypotension is low blood pressure. Blood pressure is the force of blood pushing against the walls of the arteries as the heart pumps out blood. Blood pressure is indicated by two numbers, the systolic blood pressure (the top number) and the dia ...
,
pH imbalance, and a number of other hemodynamic and blood level factors. Modeling of fluid flows to predict the likelihood of red cell membrane rupture in response to stress is an active area of research.
From bacteria culture
![Streptococcal hemolysis](https://upload.wikimedia.org/wikipedia/commons/c/cc/Streptococcal_hemolysis.jpg)
Visualizing the physical appearance of hemolysis in cultured blood samples may be used as a tool to determine the species of various
Gram-positive bacteria infections (''e.g.'', ''
Streptococcus
''Streptococcus'' is a genus of gram-positive ' (plural ) or spherical bacteria that belongs to the family Streptococcaceae, within the order Lactobacillales (lactic acid bacteria), in the phylum Bacillota. Cell division in streptococci occurs ...
'').
Nomenclature
Hemolysis is sometimes called hematolysis, erythrolysis, or erythrocytolysis. The words ''hemolysis'' ()
and ''hematolysis'' () both use
combining forms conveying the idea of "
lysis of blood" (''
hemo-'' or ''
hemato-'' + ''
-lysis''). The words ''erythrolysis'' () and ''erythrocytolysis'' ()
both use combining forms conveying the idea of "
lysis of erythrocytes" (''
erythro-'' ± ''
cyto-'' + ''
-lysis'').
Red blood cells (erythrocytes) have a short lifespan (approximately 120 days), and old (
senescent
Senescence () or biological aging is the gradual deterioration of functional characteristics in living organisms. The word ''senescence'' can refer to either cellular senescence or to senescence of the whole organism. Organismal senescence inv ...
) cells are constantly removed and replaced with new ones via
erythropoiesis
Erythropoiesis (from Greek 'erythro' meaning "red" and 'poiesis' "to make") is the process which produces red blood cells (erythrocytes), which is the development from erythropoietic stem cell to mature red blood cell.
It is stimulated by decrea ...
. This breakdown/replacement process is called erythrocyte turnover. In this sense, erythrolysis or hemolysis is a normal process that happens continually. However, these terms are usually used to indicate that the lysis is
pathological
Pathology is the study of the causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in th ...
.
Complications
Pulmonary hypertension has been gaining recognition as a complication of chronic hereditary and acquired hemolysis.
Free hemoglobin released during hemolysis inactivates the
vasodilator nitric oxide (NO).
Hemolysis also releases
arginase
Arginase (, ''arginine amidinase'', ''canavanase'', ''L-arginase'', ''arginine transamidinase'') is a manganese-containing enzyme. The reaction catalyzed by this enzyme is:
: arginine + H2O → ornithine + urea
It is the final enzyme of the ure ...
that depletes
L-arginine
Arginine is the amino acid with the formula (H2N)(HN)CN(H)(CH2)3CH(NH2)CO2H. The molecule features a guanidino group appended to a standard amino acid framework. At physiological pH, the carboxylic acid is deprotonated (−CO2−) and both the a ...
, the substrate needed for NO synthesis.
This reduces NO-dependent vasodilation
and induces
platelet
Platelets, also called thrombocytes (from Greek θρόμβος, "clot" and κύτος, "cell"), are a component of blood whose function (along with the coagulation factors) is to react to bleeding from blood vessel injury by clumping, thereby i ...
activation,
thrombin
Thrombin (, ''fibrinogenase'', ''thrombase'', ''thrombofort'', ''topical'', ''thrombin-C'', ''tropostasin'', ''activated blood-coagulation factor II'', ''blood-coagulation factor IIa'', ''factor IIa'', ''E thrombin'', ''beta-thrombin'', ''gamma- ...
generation,
procoagulant
Coagulation, also known as clotting, is the process by which blood changes from a liquid to a gel, forming a blood clot. It potentially results in hemostasis, the cessation of blood loss from a damaged vessel, followed by repair. The mechanism o ...
factors and
tissue factor activation,
contributing to the formation of
thrombosis
Thrombosis (from Ancient Greek "clotting") is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel (a vein or an artery) is injured, the body uses platelets (t ...
.
This can lead to
esophageal spasm
Esophageal spasm is a disorder of motility of the esophagus.
There are two types of esophageal spasm:
* Diffuse or distal esophageal spasm (DES), where there is uncoordinated esophageal contractions
* Nutcracker esophagus (NE) also known as hy ...
and
dysphagia
Dysphagia is difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right.
It may be a sensation that suggests difficulty in the passage of solids or liq ...
,
abdominal pain
Abdominal pain, also known as a stomach ache, is a symptom associated with both non-serious and serious medical issues.
Common causes of pain in the abdomen include gastroenteritis and irritable bowel syndrome. About 15% of people have a m ...
,
erectile dysfunction,
systemic hypertension
Hypertension (HTN or HT), also known as high blood pressure (HBP), is a long-term medical condition in which the blood pressure in the arteries is persistently elevated. High blood pressure usually does not cause symptoms. Long-term high blo ...
,
decreased organ perfusion, promotion of
inflammation
Inflammation (from la, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants, and is a protective response involving immune cells, blood vessels, and molec ...
and
coagulation
Coagulation, also known as clotting, is the process by which blood changes from a liquid to a gel, forming a blood clot. It potentially results in hemostasis, the cessation of blood loss from a damaged vessel, followed by repair. The mechanis ...
, and
thrombosis
Thrombosis (from Ancient Greek "clotting") is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel (a vein or an artery) is injured, the body uses platelets (t ...
.
Chronic hemolysis may also lead to
endothelial dysfunction
In vascular diseases, endothelial dysfunction is a systemic pathological state of the endothelium. Along with acting as a semi-permeable membrane, the endothelium is responsible for maintaining vascular tone and regulating oxidative stress by ...
, heightened
endothelin
Endothelins are peptides with receptors and effects in many body organs. Endothelin constricts blood vessels and raises blood pressure. The endothelins are normally kept in balance by other mechanisms, but when overexpressed, they contribute ...
-1-mediated responses and
vasculopathy
Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis. Vasculitis is primarily caused by ...
.
The release of
heme
Heme, or haem (pronounced / hi:m/ ), is a precursor to hemoglobin, which is necessary to bind oxygen in the bloodstream. Heme is biosynthesized in both the bone marrow and the liver.
In biochemical terms, heme is a coordination complex "consis ...
leads to the production of
bilirubin and depletion of plasma proteins, such as
albumin
Albumin is a family of globular proteins, the most common of which are the serum albumins. All the proteins of the albumin family are water-soluble, moderately soluble in concentrated salt solutions, and experience heat denaturation. Albumins ...
,
haptoglobin
Haptoglobin (abbreviated as Hp) is the protein that in humans is encoded by the ''HP'' gene. In blood plasma, haptoglobin binds with high affinity to ''free'' hemoglobin released from erythrocytes, and thereby inhibits its deleterious oxidative ...
, and
hemopexin
Hemopexin (or haemopexin; Hpx; Hx), also known as beta-1B-glycoprotein, is a glycoprotein that in humans is encoded by the ''HPX'' gene and belongs to the hemopexin family of proteins. Hemopexin is the plasma protein with the highest binding aff ...
, which may lead to
jaundice.
[ ]
Material was copied from this source, which is available under a Creative Commons License. It may also lead to increased levels of the heme breakdown product
stercobilin
Stercobilin is a tetrapyrrolic bile pigment and is one end-product of heme catabolism.Boron W, Boulpaep E. Medical Physiology: A cellular and molecular approach, 2005. 984-986. Elsevier Saunders, United States. Kay IT, Weimer M, Watson CJ (1963) ...
in the stool.
Splenectomy
A splenectomy is the surgical procedure that partially or completely removes the spleen. The spleen is an important organ in regard to immunological function due to its ability to efficiently destroy encapsulated bacteria. Therefore, removal of ...
of those with hemolytic disorders appears to increase risk of developing
pulmonary thrombosis.
Complications may also arise from the increased workload for the kidney as it secretes
erythropoietin to stimulate the
bone marrow to produce more
reticulocyte
Reticulocytes are immature red blood cells (RBCs). In the process of erythropoiesis (red blood cell formation), reticulocytes develop and mature in the bone marrow and then circulate for about a day in the blood stream before developing into ma ...
s (red blood cell precursors) to compensate for the loss of red blood cells due to hemolysis.
See also
*
Hemolysin
Hemolysins or haemolysins are lipids and proteins that cause lysis of red blood cells by disrupting the cell membrane. Although the lytic activity of some microbe-derived hemolysins on red blood cells may be of great importance for nutrient acqu ...
*
Glucose-6-phosphate dehydrogenase deficiency
Glucose-6-phosphate dehydrogenase deficiency (G6PDD), which is the most common enzyme deficiency worldwide, is an inborn error of metabolism that predisposes to red blood cell breakdown. Most of the time, those who are affected have no symptoms. ...
References
External links
{{Medical resources
, ICD10 =
, ICD9 =
, ICDO =
, OMIM =
, DiseasesDB =
, MedlinePlus =
, eMedicineSubj =
, eMedicineTopic =
, MeshID = D006461
, GeneReviewsNBK =
, GeneReviewsName =
, NORD =
, GARDNum =
, GARDName =
, Orphanet =
, AO =
, RP =
, WO =
, OrthoInfo =
, NCI =
, Scholia =
, SNOMED CT =
Effects of Hemolysis on Clinical Specimens
Hematology