What is the management approach for a 78-year-old patient with Kasabach-Merritt syndrome and Disseminated Intravascular Coagulation (DIC) defibrination syndrome presenting with a new hematoma?

Management of Kasabach-Merritt Syndrome with DIC in a 78-Year-Old Patient with New Hematoma

The primary management approach for a 78-year-old patient with Kasabach-Merritt syndrome (KMS) and DIC presenting with a new hematoma should focus on treating the underlying vascular tumor while providing supportive care for coagulopathy and bleeding complications. 1

Understanding the Condition

Kasabach-Merritt syndrome is a rare complication characterized by:

• Large vascular tumors (typically hemangiomas)
• Consumptive coagulopathy
• Thrombocytopenia
• Disseminated intravascular coagulation (DIC)

In elderly patients, KMS is particularly concerning as it carries significant mortality risk due to uncontrollable hemorrhage.

Immediate Management

1. Assessment and Stabilization

• Evaluate the extent of bleeding from the new hematoma
• Monitor vital signs for signs of hemodynamic instability
• Obtain baseline coagulation studies:
  • Platelet count
  • Prothrombin time (PT/INR)
  • Activated partial thromboplastin time (aPTT)
  • Fibrinogen level
  • D-dimer or fibrin degradation products (FDPs) 1

2. Blood Product Support

• For active bleeding or high bleeding risk:
  • Transfuse platelets if count is <50 × 10^9/L 2
  • Administer fresh frozen plasma (15-30 mL/kg) with careful monitoring 2
  • For persistently low fibrinogen (<1.5 g/L), provide cryoprecipitate or fibrinogen concentrate 2

Remember that transfused platelets and fibrinogen may have very short lifespans in DIC due to ongoing consumption 2

Treatment of Underlying Cause

Primary Treatment Approach

• Treatment of the underlying vascular tumor is the cornerstone of KMS management 2, 1
• Surgical options may be limited due to:
  • Advanced age
  • Coagulopathy
  • Size and location of tumor
  • Risk of bleeding during intervention 3

Medical Management Options

1. Systemic Glucocorticoids:

   • Initial therapy: Prednisolone 3-5 mg/kg/day 4
   • Shown effective in some adult cases of KMS 3, 5
   • Monitor for hypertension, especially in elderly patients 4

2. Anticoagulation:

   • For non-hyperfibrinolytic DIC, consider prophylactic anticoagulation in the absence of contraindications 2
   • For patients with high bleeding risk and renal impairment, unfractionated heparin (UFH) is preferred due to easier reversibility 2
   • For others, low molecular weight heparin (LMWH) is recommended 2
   • Monitor anti-Xa activity rather than PTT in DIC patients 2

3. Additional Medical Therapies:

   • Beta-blockers have shown some efficacy in combination with steroids 3
   • Sirolimus has demonstrated effectiveness in treating hemangiomas associated with KMS 5
   • Consider dipyridamole as adjunctive therapy with steroids 4
   • For severe cases, interferon-alpha-2b may be considered for steroid-resistant cases 4

4. Antifibrinolytic Therapy:

   • Tranexamic acid may be considered if therapy-resistant bleeding dominates the picture 2, 6
   • Not recommended for routine use in all DIC cases 2
   • Has shown efficacy when combined with anticoagulants like danaparoid in some cases 6

Management of Complications

For New Hematoma

• Apply local pressure if accessible
• Avoid unnecessary invasive procedures
• If surgical drainage is necessary, correct coagulopathy before procedure
• Choose compressible sites only for any necessary vascular access 1

For Thrombotic Complications

• For patients developing arterial or venous thrombosis, therapeutic-dose anticoagulation should be used 2
• In patients with severe thrombocytopenia (<25-50 × 10^9/L) and new thrombus, consider:
  • Platelet transfusions with therapeutic anticoagulation
  • Intermediate-dose or prophylactic anticoagulation without transfusions
  • No anticoagulation if thrombus is not in a critical location 2

Ongoing Monitoring

• Regular clinical and laboratory surveillance to assess:
  • Improvement or worsening of coagulopathy
  • Development of complications including organ failure
  • Response to treatment of underlying condition 2, 1
• Serial laboratory testing (every 6-12 hours initially):
  • Platelet count
  • PT/INR
  • Fibrinogen
  • D-dimer 1

Pitfalls to Avoid

1. Overlooking the short lifespan of transfused products in active DIC 2
2. Misinterpreting normal coagulation screens (normal PT/aPTT does not exclude DIC) 1
3. Using antifibrinolytic agents without clear indication of hyperfibrinolytic DIC 2
4. Using recombinant Factor VIIa, which carries thrombotic risks and lacks evidence in KMS-associated DIC 2
5. Delaying treatment of the underlying vascular tumor 2, 3

Prognosis

KMS with DIC in elderly patients carries significant mortality risk, especially with giant vascular tumors. Early intervention focusing on both the underlying tumor and the coagulopathy is essential to improve outcomes 3, 7.