One of the rare and weird things in medicine that combine a dramatic constellation of opposites. Caring for someone in the throes of badness from two dichotomous problems leaves one in a state of floundering. Do I treat the clotting or do I treat the bleeding? What will kill them first? It’s universally known that getting the diagnosis of disseminated intravascular coagulation is a dreaded event. DIC is a downstream complication that arises late in the game with several diagnoses that we see in the ICU, and unfortunately in our maternal population. Every time an OB calls our team to see or transfer a patient to ICU my shoulders instantly feel tense. Let’s talk about the pathophysiology behind this dreaded state and what our treatment options really are.

Etiology

An insult occurs which causes the release of cytokines.

• Infection: the body starts communicating the need to fight which it does via cytokine release.

• Infection: bacteria release endotoxins (esp gram negative bacteria).

• Trauma: accidental or surgical damage to the endothelial cells cause the release of cytokines. Orthopedic trauma can also cause release of fat (bone fx) and lipids into bloodstream.

• Certain types of hemolysis: trauma, autoimmune hemolytic anemia, blood transfusion reaction, snake venom, HELLP.

• Cancer: especially leukemia. This pathway is actually different. Cancer (solid organ and hematologic) stimulates the coagulation pathway via TF release and direct pro-coagulant factors produced by the tumor cells. leading to consumption. Tends to be a more gradual, less dramatic progression.

• SIRS: non infectious mediated endothelial damage induced by issues like burns, pancreatitis.

• OB emergencies: pre-eclampsia (abnormal development of placental blood vessels), amniotic fluid embolism, intrauterine death, retained fetal products, placental abruption, post-partum hemorrhage. The placenta regulates coagulation pathways to maintain hemostasis and when damage occurs here it can spill over into the systemic circulation. Tissue factor (a cytokine) release is the primary pathway.

• Direct endovascular injury: aortic aneurysm, cavernous hemangioma.

• ECMO - consumption of coag factors, thrombocytopenia possibly acquired von Willebrand’s, fibrinolysis promotion.

• Transplant rejection: coagulation cascade activation can occur as late stage rejection leading to fibrin deposits in the bloodstream.

Pathway

Any of the above can lead to dysregulation of the coagulation pathway. It starts with widespread endovascular clot formation which uses up the body’s supply of coagulation factors. This then creates an environment for bleeding as there is a shortage of clotting factors and thrombocytopenia (from consumption).

Cytokine activity -> fibrin accumulation (low fibrinogen levles/high fibrinogen degradation products) -> lots of small endovascular clots -> tissue ischemia (multi-system organ failure).

Cytokine activity -> fibrin accumulation -> clots -> low platelets (consumed) + coagulopathy -> bleeding .

Diagnosis

1. Clinical scenario that supports the diagnosis.

2. Signs/Symptoms: bleeding, DVT’s/PE formation, stroke symptoms (if neuro thrombus), cardiac symptoms, bruising, petechiae.

3. Labs:

   • Platelets - low (in early stage may be low normal).

   • Fibrinogen - low (in early stage may be low normal).

   • Fibrinogen degrading products - high.

   • PT/aPTT - prolonged (but may be normal in hemolytic anemia).

   • D-Dimer - elevated (indirect measure of fibrin formation - many things can elevate this).

   • TEG- MA <60mm (strongest indicator; 73% specificity), K> 2m, wine glass will look overall skinnier and longer (champagne flute).

4. ISTH score uses some labs and clinical indicator to spit out a score. If >5 suggests overt DIC.

Treatment

Unfortunately, this is the frustrating part. DIC is always a secondary insult so it will not correct until you fix the primary cause. If you look at the list of the causes above, some are correctable and some are not. Your immediate management will come down to factor repletion. This will provide a band aid.

• Factor repletion as indicated. I like to use TEG as a guideline.

  • Long R time / elevated INR / elevated aPTT: FFP.

  • Low platelets / low MA: Platelets; consider DDAVP.

  • Long K time / low fibrinogen / low alpha angle: Cryoprecipitate.

  • Low hemoglobin: PRBC.

  • High DD, evidence of clotting without bleeding or stable/chronic DIC: consider heparin.

• Corrections:

  • OB: deliver the baby, remove the placenta or retained fetal products. Mitigate pre-eclampsia.

  • ECMO: stop ECMO - if you can.

  • SIRS/Sepsis: find the source, treat the cause appropriately. SIRS may be harder.

  • Trauma: stop the bleed, stitch up the injury, pray you don’t develop ARDS.

  • Transfusion reactions: stop the transfusion, treat the TRALI that may develop.

  • Autoimmune hemolytic anemia - you may be able to consider IVIG - talk to a hematologist.

  • Snake Envenomation: antivenom, time.

  • Transplant rejection: transfer to a transplant center.

  • Cancer: can you treat the cancer? If not ICU level sick possibly chemo, surgery, radiation.