Disseminated intravascular coagulation: Causes, Symptoms, Treatment and Prevention

Disseminated intravascular: Normal blood coagulation refers to mechanism of blood clotting in our body.  However, there are haemostatic changes in pregnancy which may impact on maternal coagulation. These changes in haemostasis occur during pregnancy are:

  • Increase blood fibrinogen level
  • Increase blood volume
  • Increase susceptibility of intravascular coagulation during pregnancy

All these help in maintaining haemostasis after normal delivery

  • There is increase in local platelet response leading to formation of small fibrin clots and removal of unwanted fibrin by fibrinolysis.
  • There is activation of factor VII by thromboplastin (extrinsic factor) when there is damage vessel.
  • Thromboplastin converts prothrombin to thrombin in the presence of calcium ions
  • Thrombin then converts fibrinogen to fibrin. The fibrin forms a mesh network that entraps blood cells and clot is formed. This involves large number of clotting factors. In order for main circulation of blood as fluid in the body system fibrinolysis takes place which is breakdown of fibrin. If fibrinolysis did not occur, coagulation will continue.

What is Disseminated Intravascular Coagulation (DIC)?

DIC is a condition that involves formation of clots in circulation usually secondary to stimulation of coagulation system leading to consumption of clotting factors. As a result of this, there is failure of failure of clot at bleeding site.

DIC encompasses the term used in describing patient with excessive coagulation, bleeding, thrombus formation, infarction, fibrinolysis and consumption/destruction of platelets and plasma factors.

Disseminated intravascular coagulation, otherwise called DIC, hypofibrinogenaemia, consumptive coagulopathy, Dysfibrinogen syndrome or thrombohemorrhagic syndrome, is a blood coagulation failure which may be caused by sepsis, trauma, malignancy, and obstetric complications.

Compbell (2006) defined DIC as acquired disorders of blood clotting. DIC can be caused by massive and inappropriate inflammatory response due to traumatic or septic conditions (2).

What are the causes of disseminated intravascular coagulation?

DIC is caused by a number of obstetric conditions such as:

  • Premature separation of the placenta(placenta abruption)
  • Pregnancy-induced hypertension
  •  Amniotic fluid embolism,
  • Placenta retention
  •  Septic abortion
  • Neonatal hypoxic-ischemic encephalopathy syndrome
  • IUD/ Retention of a dead fetus(missed abortion/ delayed miscarriage)
  • Intrauterine infection (e.g. septic abortion)
  • local infections (endometritis)
  • Pre-eclampsia and eclampsia
  • Haemorrhage and shock

Other causes of DIC/thrombohemorrhagic syndrome are:

  • Neoplasia/cancer, pancreatitis and  liver disorders
  • endotoxemia /systemic sepsis(e.g. viruses, Rickettsiae)
  • inflammation from an injury/ trauma, infection and/or illness
  • heatstroke and envenomation
  • gastrointestinal disease (large colon volvulus, colitis)
  • immune-mediated disease (purpura hemorrhagica)

Note 

Thesedisorders above trigger systemic inflammation that causes DIC.

Parasites like plasmodium falciparum can cause DIC but often cause by Gram-negative bacterial infections and sometimes by Gram-positive sepsis.

Who are higher at risk of Hypofibrinogenaemia/ Dysfibrinogen Syndrome?

  • Those with sepsis (an infection in the blood stream)
  • Those with surgery and trauma (e.g. polytrauma, neurotrauma or fat embolism)
  • Those with cancer, pancreatitis and  liver disorders
  • Those with serious complications of pregnancy and childbirth

What are types of DIC?

DIC can be classified based on duration and degree/ severity

Based on duration:

  • Acute DIC:  Here, its onset is sudden, characterized by bleeding symptoms and it can be hours or days. It requires quick treatment if patient’s life is to be saved. Deficient factor activity is best replaced with plasma infusions alongside with cryoprecipitate to further replete fibrinogen.
  • Chronic DIC: It develops gradually and it can take weeks or months. Treating the underlying cause would stop the development of DIC. That is only effective long-term treatment of chronic DIC.

Based on degree/severity:

  • Mild DIC: When it is tolerable and can easily be managed. The patient’s condition has not deteriorated. Low dose of heparin can be administered. Heparin (an anticoagulant) works by inhibiting the activation of coagulation system and in turn providing anti-inflammatory and immunodulatory function.
  • Moderate DIC: When patients’ blood is moderately affected and can be treated just like in mild DIC.
  • Severe DIC: This is the highest form of DIC; the patient is highly endangered and death may be inevitable.

What are signs and symptoms of Hypofibrinogenaemia?

The classic signs and symptoms of DIC include:

  • Redness, warmth, pain and swelling in the lower leg particularly if blood clots develop in the deep veins of the legs
  • Headaches and speech changes(e.g. trouble speaking)
  • Paralysis(inability to move)
  • Bleeding from various sites in the body
  • Bruising and haematuria(blood in urine)
  • Sudden drop in blood pressure
  • Pyrexia/fever
  • Shortness of breath
  • Memory loss, confusion or changes in behaviour

Note: These signs above may also denote stroke.

What are warning signs of disseminated intravascular coagulation?

One of the key warning signs of disseminated intravascular coagulation is easy bruising or bleeding from an intravenous site.

Pathophysiology of DIC/ Dysfibrinogen syndrome

DIC is not a primary disease but always associated with another condition that triggers formation of microthrombi throughout the circulation. There is consumption of clotting factors. DIC triggers fibrinolysis and production of fibrin degradation products (FDPs) resulting in severe haemorrhage.

Clotting then becomes a problem (inability of clotting to occur). FDPs inhibit myometrial activities leading to inability of myometrium to contract after birth.

The open vessels I the placental site remains open and cannot be constricted due to myometrial dysfunction. Visible blood loss occurs and this blood remains unclotted, even if clots occur it is unstable.

The microthrombi equally affect the contractility of the heart, block tiny vessels leading to cardiovascular accident (CVA) and organ failures such as liver and kidneys.

 What are stages of DIC?

DIC occurs in four stages or phase but in continuous process and overlapping each other as follows:

  • First stage of DIC: The disorder is just starting but it’s also life-threatening. This stage is also called hypercoagulation but it is not noted clinically. A low-dose heparin can improve the condition of patients suffering from early disseminated intravascular coagulation (pre-DIC).
  • The second stage of DIC: This is when there is haemorrhage caused by disorders in fibrin production. It is also called compensated or subclinical stage and usually there are alterations in coagulation profiles or end-organ dysfunction.
  • In third stage of DIC syndrome: There is repeated hypercoagulation, suppressed fibrinolysis, and microthrombolysis in organs and tissues resulting in multiple organ failure. This stage is otherwise called fulminant or uncompensated stage because fulminant coagulopathy and signs of hemorrhage are common.
  • The fourth stage of DIC: There aremultiple organs and generalized inflammation accompany with 3-5 days and later haemorrhages.  This is the end-stage DIC and death is inevitable.

Investigations

  1. Clotting time is carried out
  2. Platelets count
  3. FBC and blood grouping and crosshatching

How to manage Disseminated Intravascular Coagulation

The objectives of managing DIC are:

  1. To treat the underlying cause and remove stimulus-provoking DIC
  2. To ensure maintenance of circulating blood volume
  3. To replace consumed clotting factors and destroyed red blood cells
  4. The midwife should be knowledgeable about conditions that cause DIC
  5. She should be able to recognize signs of DIC e.g., failure of injection site to stop bleeding, bleeding from mucous membrane
  6. Frequent and accurate observations

The best treatment for DIC

This includes:

  • Transfusion of fresh whole blood or fresh frozen plasma
  • Fibrinogen or triple strength plasma transfusion
  • Give oxygen and resuscitate with IV drip
  • Drugs as prescribed, e.g. Morphine for pain

Intravenous (IV) Syntocinon if uterus is lax or not contracted.

What medication is good for DIC?

Heparin is only useful when circulation is intact, but in case of DIC it worsens the situation. Use of anti-fibrinolytic agents also precipitate blockage of small vessels especially that of the kidneys and heart.

However, Apoprotonin (an anti-fibrinolytic and anti-coagulant) can be used especially with severe bleeding from placental site. It has also been recommended in cases of placenta abruption with uterine inertia.

The patient will respond quickly to this treatment if given quickly.  Advise facility-based (hospital) delivery for the next time and warm her to explain to doctor or nurse. It is important to be able to differentiate between a tonic and traumatic postpartum haemorrhage.

Midwives’ Roles in managing DIC/ Hypofibrinogenaemia

  • Prompt recognition of the situation and prompt action.
  • The midwife must maintain calmness and critical thinking in order to help couple deal with the situation.
  • Frequent and accurate observations must be maintained to keep view on, mothers’ condition. This includes: vital signs, general condition and fluid balance (for signs of renal failure).
  • Appropriate     attention should be given to the partner and other     family members

What are complications of DIC?

Poorly managed consumptive coagulopathy/DIC results in these complications:

  • Total derangement of hemostasis(uncontrollable haemorrhage)
  • Multiple organ failure
  • Other haemologic disorders (e.g. thrombotic phenotype or a hemorrhagic phenotype)
  • Maternal death

What is prognosis of Hypofibrinogenaemia?

Most of DIC cases result in maternal death if prompt action was not taken to ameliorate the situation (4). However, where there is prompt action and resuscitative equipment, the mother is far more likely to survive.

How to prevent consumptive coagulopathy (DIC)

No specific measures are available for the prevention of DIC. However, patients suffering from cancer, pancreatitis, liver disorders and other debilitating medical conditions can receive a prophylactic low-dose heparin because it helps to improve the hypercoagulable state of sepsis and lower the incidence of   DIC and/ or multiple organ dysfunction syndromes as well as the number of days of mechanical ventilation and hospitalization(1).

Reference

  1. Liu, X. L., Wang, X. Z., Liu, X. X., Hao, D., Jaladat, Y., Lu, F., Sun, T., & Lv, C. J. (2014). Low-dose heparin as treatment for early disseminated intravascular coagulation during sepsis: A prospective clinical study. Experimental and therapeutic medicine, 7(3), 604–608. https://doi.org/10.3892/etm.2013.1466
  2. Blaisdell FW.( 2012).Causes, prevention, and treatment of intravascular coagulation and disseminated intravascular coagulation. J Trauma Acute Care Surg. 2012 Jun;72(6):1719-22.https://www.doi.org/ 10.1097/TA.0b013e3182444491
  3. Clinical Veterinary Advisor (2012). Disseminated Intravascular Coagulation. The Horse2012, Pages 152-154; https://doi.org/10.1016/B978-1-4160-9979-6.00103-3

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