How should disseminated intravascular coagulation be managed?

Management of Disseminated Intravascular Coagulation

The cornerstone of DIC management is aggressive treatment of the underlying trigger (sepsis, malignancy, trauma, obstetric complications), combined with phenotype-specific supportive care: transfusion support for bleeding-predominant DIC and therapeutic anticoagulation for thrombosis-predominant DIC. 1, 2

Identify and Treat the Underlying Cause

• Immediate source control is mandatory: administer appropriate antibiotics for sepsis, initiate chemotherapy for malignancy (especially acute promyelocytic leukemia where early treatment achieves rapid DIC resolution), perform surgical intervention for trauma, or proceed with delivery for obstetric complications. 1, 2

• DIC will not resolve without addressing the root cause, making this the primary therapeutic goal that supersedes all supportive measures. 1, 2

Classify the Clinical Phenotype

DIC presents in three distinct forms that dictate management strategy 3, 2:

Procoagulant (Thrombosis-Predominant) DIC

• Common in pancreatic cancer and adenocarcinomas. 3
• Presents with arterial ischemia (patchy skin discoloration, digital ischemia, stroke), venous thromboembolism, or microvascular thrombosis. 3
• Treatment priority: therapeutic anticoagulation. 1, 2

Hyperfibrinolytic (Bleeding-Predominant) DIC

• Typical of acute promyelocytic leukemia and metastatic prostate cancer. 3
• Presents with widespread bruising, mucosal bleeding, CNS hemorrhage, or gastrointestinal bleeding. 3
• Treatment priority: aggressive transfusion support; avoid heparin. 1, 2

Subclinical DIC

• Laboratory abnormalities only (thrombocytopenia, hypofibrinogenemia, elevated D-dimer) without overt bleeding or thrombosis. 3
• May progress to overt DIC if the underlying condition is not treated. 2, 4

Transfusion Support for Bleeding-Predominant DIC

Platelet Transfusion

• Maintain platelets >50 × 10⁹/L in actively bleeding patients. 1, 2
• For high-risk patients without active bleeding: transfuse if platelets <30 × 10⁹/L in acute promyelocytic leukemia or <20 × 10⁹/L in other malignancies. 1, 2
• Recognize that transfused platelets have a very short lifespan in DIC due to ongoing consumption, often requiring repeated dosing. 1, 2

Fresh Frozen Plasma

• Administer 15–30 mL/kg to bleeding patients with prolonged PT/aPTT. 1, 2
• Do not transfuse based solely on laboratory abnormalities; clinical bleeding or high procedural risk must drive the decision. 1, 5
• If volume overload is a concern, prothrombin complex concentrates may be used, though they provide only selected clotting factors and do not fully correct the global deficiency. 1, 5

Fibrinogen Replacement

• Administer two pools of cryoprecipitate or fibrinogen concentrate if fibrinogen remains <1.5 g/L despite plasma support in actively bleeding patients. 1, 2, 5
• Cryoprecipitate should not be given to patients with laboratory evidence of DIC but no active bleeding. 1

Anticoagulation for Thrombosis-Predominant DIC

Indications for Therapeutic Anticoagulation

• Start therapeutic-dose heparin (preferably low-molecular-weight heparin) for arterial/venous thromboembolism, severe purpura fulminans with acral ischemia, vascular skin infarction, or cancer-associated DIC with documented thrombotic events. 1, 2, 4, 5

• Prolonged PT/aPTT alone should not preclude anticoagulation in the absence of bleeding, as DIC reflects a rebalanced hemostatic state with simultaneous loss of pro- and anticoagulant factors. 1, 4

Choice of Anticoagulant

• Low-molecular-weight heparin is preferred in most cases. 1, 2
• Unfractionated heparin is preferred for patients at high bleeding risk with renal impairment due to its rapid reversibility and short half-life. 1, 2

Prophylactic Anticoagulation

• In solid-tumor-associated DIC without active bleeding, consider prophylactic heparin if platelets ≥20 × 10⁹/L and no active bleeding is present. 1, 2
• In critically ill, non-bleeding patients with DIC, prophylactic doses of heparin or low-molecular-weight heparin are recommended for venous thromboembolism prevention. 5

Critical Contraindication

• Heparin must be avoided in hyperfibrinolytic DIC, where it can exacerbate bleeding. 1, 2

Agents to Avoid

• Tranexamic acid should not be used routinely in DIC and may increase thrombotic events; reserve it only for therapy-resistant bleeding with documented hyperfibrinolysis. 1, 2, 5

• Recombinant Factor VIIa is not recommended because its benefit is uncertain and it carries a definite thrombotic risk. 1, 2

• Corticosteroids have no established benefit and are not recommended by major DIC guidelines. 1

• Antiplatelet agents are not indicated and may increase bleeding risk. 1

Monitoring Strategy

• Monitor CBC, PT/aPTT, fibrinogen, and D-dimer daily in acute DIC with active bleeding or rapid deterioration. 2, 4

• Adjust monitoring frequency based on clinical stability: every 2–3 days for stable ICU patients, weekly for hospitalized patients with chronic cancer-associated DIC, and monthly for outpatients with stable subclinical DIC. 4

• A ≥30% drop in platelet count may indicate subclinical DIC progression, even if the absolute count remains normal. 2, 4

• Dynamic trend analysis over hours to days is more diagnostically important than single absolute values. 4

Common Pitfalls

• Do not transfuse solely based on laboratory abnormalities; clinical bleeding or procedural risk must drive transfusion decisions. 1, 5

• Do not withhold anticoagulation solely because of prolonged PT/aPTT in thrombotic DIC; the hemostatic balance in DIC permits anticoagulation when bleeding is absent. 1, 4

• Recognize that transfused blood products have reduced survival in active DIC, often requiring repeated administrations to maintain hemostatic targets. 1, 2

• Do not use antifibrinolytics routinely; they should be reserved for refractory bleeding with documented hyperfibrinolysis. 1, 2, 5