How should disseminated intravascular coagulation be treated?

Treatment of Disseminated Intravascular Coagulation

The cornerstone of DIC treatment is aggressive management of the underlying disorder, with simultaneous supportive hemostatic therapy guided by bleeding status and specific laboratory thresholds, not laboratory abnormalities alone. 1

Immediate Identification and Treatment of Underlying Cause

The first priority is identifying and treating the trigger mechanism, as DIC will not resolve without addressing the root cause 1, 2:

• Sepsis: Initiate source control and appropriate antibiotics immediately 1, 2
• Malignancy: Begin cancer-directed therapy (chemotherapy, surgery, or radiation) without delay 2
• Acute promyelocytic leukemia: Start all-trans retinoic acid emergently, which achieves excellent resolution of the coagulopathy 2
• Trauma: Surgical intervention as indicated 1
• Obstetric complications: Proceed with delivery and manage eclampsia 1

Classification of DIC Phenotype (Critical for Treatment Decisions)

DIC presents in three distinct forms that require different management approaches 1, 2:

Procoagulant DIC (Thrombosis-Predominant)

• Common in pancreatic cancer and adenocarcinomas 2
• Presents with arterial ischemia, venous thromboembolism, or microvascular thrombosis 2
• Requires anticoagulation 1, 2

Hyperfibrinolytic DIC (Bleeding-Predominant)

• Typical of acute promyelocytic leukemia and metastatic prostate cancer 2
• Presents with widespread bleeding from multiple sites 2
• Anticoagulation is contraindicated 1, 2

Subclinical DIC

• Laboratory abnormalities without overt clinical manifestations 1
• Diagnosed by ≥30% drop in platelet count, even when absolute values remain normal 1, 2

Supportive Hemostatic Management

For Active Bleeding

Maintain platelets >50×10⁹/L through platelet transfusions 1, 2

Administer fresh frozen plasma (FFP) 15-30 mL/kg for prolonged PT/aPTT 1, 2

Replace fibrinogen if <1.5 g/L persists despite FFP using cryoprecipitate or fibrinogen concentrate 1, 2

A critical caveat: transfused platelets and fibrinogen have very short lifespans in DIC with vigorous coagulation activation, often necessitating repeated transfusions 1. This reality must inform goals of care discussions.

For High Bleeding Risk Without Active Hemorrhage

• Transfuse platelets if <30×10⁹/L in acute promyelocytic leukemia 2
• Transfuse platelets if <20×10⁹/L in other cancer types 2

Anticoagulation Strategy

Prophylactic Anticoagulation

Initiate prophylactic anticoagulation with heparin in all patients with cancer-related DIC EXCEPT those with hyperfibrinolytic DIC, unless contraindications exist (platelets <20×10⁹/L or active bleeding) 3, 2

• Low molecular weight heparin (LMWH) is the first choice for most patients 2
• In patients with renal impairment at high bleeding risk, use unfractionated heparin instead because it can be more readily reversed 1

Therapeutic Anticoagulation

Escalate to therapeutic-dose anticoagulation if any of the following develop 3, 1:

• Arterial or venous thromboembolism
• Severe purpura fulminans with acral ischemia
• Vascular skin infarction
• Cancer-associated DIC with thrombotic events

Special Scenario: Thrombosis with Severe Thrombocytopenia (<25-50×10⁹/L)

Three possible approaches exist 3:

1. Platelet transfusions plus therapeutic anticoagulation
2. Intermediate-dose or prophylactic anticoagulation without transfusions
3. No anticoagulation unless the thrombus site is critical (e.g., pulmonary embolism vs. deep vein thrombosis)

The decision depends on bleeding risk versus thrombotic risk at the specific anatomic location.

Agents to Avoid

Do not use tranexamic acid routinely in DIC 3, 1. It may increase thrombotic events and is reserved ONLY for therapy-resistant bleeding in hyperfibrinolytic DIC 3, 1. Even in acute promyelocytic leukemia, systematic tranexamic acid prophylaxis showed no clear advantage in reducing hemorrhagic incidents and trended toward higher thrombotic events 3.

Do not use recombinant factor VIIa 3, 1. Its benefit is uncertain and it carries definite thrombotic risks 3, 1.

Avoid inferior vena cava filters except in highly selected cases: Only consider temporary filters in patients who cannot be anticoagulated but have proximal lower limb thrombosis likely to embolize 3, 2. In other situations, filters can be deleterious by further activating the coagulation system 3.

Monitoring Strategy

Monitor CBC, PT/aPTT, fibrinogen, and D-dimer with frequency adjusted to clinical severity 1, 2:

• Daily in acute severe DIC 1, 2
• Less frequently as stability improves 1, 2
• Monthly in chronic stable DIC 1, 2

Watch for a 30% drop in platelet count, which may indicate subclinical DIC progression even if the absolute count remains normal 1, 2.

Common Pitfalls

Laboratory abnormalities alone should not drive transfusion decisions in the absence of bleeding or planned procedures 1. Conversely, coagulation abnormalities should not be considered absolute contraindications to anticoagulation when thrombosis is the dominant clinical feature 4.

In patients with do-not-resuscitate orders, recognize that repeated blood product transfusions provide only short-lived correction and may not align with comfort-focused goals of care 1.