What are the other Names for this Condition? (Also known as/Synonyms)

• Consumption Coagulopathy
• Defibrination Syndrome
• DIC (Disseminated Intravascular Coagulation)

What is Disseminated Intravascular Coagulation? (Definition/Background Information)

• Disseminated Intravascular Coagulation (DIC) is a serious and potentially life-threatening condition, in which blood clots form in small blood vessels throughout the body. This leads to disrupted blood flow, followed by excessive bleeding. Typically, the disorder occurs as a manifestation of an underlying medical condition
• Disseminated Intravascular Coagulation can be acute or chronic. The acute form develops over a few hours to a few days, whereas chronic DIC occurs slowly; it can take several weeks to months for symptoms to appear
• The disorder can affect male and female individuals of all ages. Infection of blood, liver disease, pancreatitis, and certain types of cancer are some risk factors for Disseminated Intravascular Coagulation
• Certain biological or infectious agents may get discharged into blood, because of an underlying medical condition. This may stimulate excessive clotting, using up the clotting factors and blood platelets. This depletion can cause excessive bleeding in the small blood vessels
• Bruises, internal and external bleeding from multiple sites in the body, and a decrease in blood pressure are the characteristic symptoms of Disseminated Intravascular Coagulation, which may require blood tests for an accurate diagnosis
• A treatment for Disseminated Intravascular Coagulation is based on its underlying cause. Blood thinners and plasma transfusions are generally the treatment recommendations
• The outcome of Disseminated Intravascular Coagulation is determined by the severity of symptoms and the underlying cause. In some cases, DIC can cause internal organ damage and may be a fatal condition

Who gets Disseminated Intravascular Coagulation? (Age and Sex Distribution)

• Disseminated Intravascular Coagulation can affect individuals of all ages and both genders
• No ethnic or racial predilection is reported for the occurrence of DIC

What are the Risk Factors for Disseminated Intravascular Coagulation? (Predisposing Factors)

The following are some known risk factors that can contribute to the development of Disseminated Intravascular Coagulation:

• Bacterial or fungal infection of blood
• A reaction to blood transfusion
• Severe tissue injury due to burns or trauma
• Surgery in the recent past
• Anesthesia
• Complications in pregnancy including pre-eclampsia and abruptio placenta (the separation of placental lining from mother’s uterus before delivery)
• Miscarriage, with some of the fetal tissue remaining inside the body
• Liver diseases
• Pancreatitis or inflammation of the pancreas
• Certain types of cancer (such as leukemia)
• Cerebral malaria
• Presence of hemangiomas (malformed blood vessels)
• Aortic aneurysm
• Certain genetic disorders, such as protein C or S deficiencies (proteins C and protein S are part of the body’s anti-coagulant system)
• Snake bites
• Frostbite

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Disseminated Intravascular Coagulation? (Etiology)

Disseminated Intravascular Coagulation is caused as a consequence of an underlying medical condition, such as an infection, inflammation, or certain types of cancer.

• Under normal circumstances, the clotting of blood (or coagulation) is a response to stop injured blood vessels from bleeding
  • This process involves the narrowing of blood vessels, mobilization of platelets to the site of injury to form plugs, activation of specific proteins in blood (such as activation of prothrombin to thrombin), and conversion of fibrinogen to fibrin, which forms the mesh-like clot
  • The event occurs in an orderly fashion (known as the coagulation cascade), with several coagulation factors activated in a specific order to bring about clotting
• When clotting is no longer required, the regulatory machinery of the body consisting of anti-coagulant factors (i.e., the C and S proteins) control or reverse clotting
• An infection, inflammation, or disease can release chemical messengers in blood that interfere with the coagulation cascade and/or anti-coagulation regulatory mechanism. This can lead to:
  • Hyper-coagulation inside the tiny blood vessels in the organs. These clots block blood supply to organs such as the liver, kidneys, or brain, which can damage the organs
  • The increased clotting can utilize most of the available factors of the coagulation cascade, rendering an individual vulnerable to excessive bleeding even with a minor injury
  • Additionally, bleeding can occur without an injury (spontaneous), both inside and outside the body

Certain inherited disorders, such as deficiency in protein C and protein S (anti-coagulation factors) can also cause DIC. In newborns, a condition known as “purpura fulminans” is caused by such inherited disorders that are characterized by purple skin lesions on the body.

• Protein C deficiency:
  • PROC gene codes for protein C, which is present in the bloodstream, and functions by deactivating specific proteins that promote clotting
  • Mutation(s) in PROC gene can lead to reduced levels of protein C (type I) or synthesis of an altered and dysfunctional form of the protein (type II). Both types lead to problems with regulation of the clotting process
  • The disorder is inherited in an autosomal dominant manner, and may be mild or severe, depending on the number of altered copies an individual inherits from his/her parent(s)
• Protein S deficiency:
  • The PROS gene codes for protein S. This protein is a co-factor of protein C, and functions in the blockage of proteins involved in the clotting process
  • Mutation(s) in the PROS gene cause 3 subtypes of deficiency, all leading to reduced levels of functional protein S, and a consequent increase in coagulation
  • Protein S deficiency is inherited in an autosomal dominant manner, and symptoms may range from mild to severe, depending on whether an individual inherits altered gene copies from one or both parents

In protein C and protein S deficiency, if parents have different types of mutation in the causative gene, their offspring may inherit both mutations, causing severe deficiency in protein C or protein S. The parents, owing to a single mutation in their genetic make-up, may have milder symptoms compared to their children.

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring.

What are the Signs and Symptoms of Disseminated Intravascular Coagulation?

The signs and symptoms of Disseminated Intravascular Coagulation may be absent, mild, or severe, and can develop rapidly or over a period. The following are common signs and symptoms of DIC:

Acute signs and symptoms:

• Uncontrolled bleeding, after delivery of a baby or surgery
• Internal bleeding
• Bleeding in body cavities
• Drop in blood pressure

Chronic signs and symptoms:

• Blood clots in veins (may be in cancer patients), particularly in the legs, presenting as swelling, redness, or pain in the affected area
• Clots that form, may travel to the lungs causing shortness of breath
• Bruising
• Drop in blood pressure

How is Disseminated Intravascular Coagulation Diagnosed?

The diagnosis of Disseminated Intravascular Coagulation is made based on the following tests and exams:

• Complete physical examination and assessment of symptoms
• Evaluation of family medical history
• Blood tests for the following:
  • Complete blood count
  • Measurement of fibrinogen
  • Partial thromboplastin time (PTT) test, to determine time taken for blood to clot
  • Prothrombin time (PT) test, to determine time taken for the liquid portion of blood to clot
  • Fibrin fibrinogen degradation product test, to measure plasma D-dimers, which can help confirm hypercoagulation

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Disseminated Intravascular Coagulation?

Some potential complications of Disseminated Intravascular Coagulation include:

• Excessive bleeding
• Damage to internal organs owing to decreased blood flow
• Reduced blood flow to the arms and legs
• Stroke

How is Disseminated Intravascular Coagulation Treated?

The treatment for Disseminated Intravascular Coagulation is generally based on each specific case; by addressing the underlying medical condition that initiated DIC. The following supportive methods may be employed:

• For excessive bleeding: Transfusions of plasma may be performed to increased the clotting factors in blood and stem bleeding
• For excessive clotting: Blood thinner medications, such as heparin, may be recommended (typically in those with milder chronic symptoms)

How can Disseminated Intravascular Coagulation be Prevented?

Disseminated Intravascular Coagulation may be preventable, if the individual seeks prompt treatment for the medical condition that can potentially cause DIC.

• If the disorder is caused by a gene mutation that runs in the family, then genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• In such cases, genetic counseling will also help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders such as DIC

Regular medical screening at periodic intervals with tests and physical examinations are strongly recommended.

What is the Prognosis of Disseminated Intravascular Coagulation? (Outcomes/Resolutions)

• The prognosis of Disseminated Intravascular Coagulation depends on the underlying cause of the disorder, and whether DIC is acute or chronic
• Identifying the medical condition causing the disorder (such as infection, inflammation, obstetric complications, or cancer), and seeking prompt treatment for the same, may lead to positive outcomes
• If DIC develops suddenly, the condition can be life-threatening and may result in fatalities

Additional and Relevant Useful Information for Disseminated Intravascular Coagulation:

Snakebites cause a condition known as “venom-induced consumption coagulopathy” or VICC. Although the symptoms may be similar, some researchers contend that a snake bite does not cause systemic microvascular thrombosis and end-organ failure, and therefore, should not be categorized as Disseminated Intravascular Coagulation.