From the Guidelines
Late-stage Purpura Fulminans (PF) treatment should prioritize anticoagulation plus protein C replacement to reduce morbidity and mortality, as suggested by the most recent guidelines. The management of late-stage PF requires a comprehensive approach to address the underlying cause of the condition, which is often related to protein C deficiency. According to the American Society of Hematology 2018 guidelines for management of venous thromboembolism: treatment of pediatric venous thromboembolism 1, the use of anticoagulation plus protein C replacement is recommended over anticoagulation alone in pediatric patients with congenital purpura fulminans due to homozygous protein C deficiency.
Key Components of Treatment
- Replacement of protein C concentrate (60-80 IU/kg initial dose followed by 40-60 IU/kg every 6-8 hours) is essential if protein C deficiency is the underlying cause 1.
- Anticoagulation therapy, such as unfractionated heparin at 10-15 units/kg/hour, should be initiated to prevent further thrombosis.
- Fresh frozen plasma (10-15 mL/kg) can be used if protein C concentrate is unavailable.
- Broad-spectrum antibiotics (such as vancomycin 15-20 mg/kg IV q8-12h plus meropenem 1-2g IV q8h) may be necessary if an infectious etiology is suspected.
- Supportive care in an intensive care setting, including inotropic support, mechanical ventilation, and renal replacement therapy, may be required for organ dysfunction.
Rationale for Treatment
The pathophysiology of PF involves uncontrolled activation of coagulation pathways leading to microvascular thrombosis, so treatment aims to interrupt this process even in late stages. The use of anticoagulation plus protein C replacement has been shown to be effective in reducing the risk of further thrombosis and improving outcomes in patients with PF 1. While protein C replacement may be expensive, the benefits of this treatment approach outweigh the costs in terms of reducing morbidity and mortality.
Considerations for Treatment
It is essential to note that the treatment approach may vary depending on the underlying cause of PF and the patient's individual needs. However, the cornerstone of management should always prioritize anticoagulation plus protein C replacement to reduce morbidity and mortality. As suggested by the American Society of Hematology 2018 guidelines 1, protein C replacement may be superior to anticoagulation alone in pediatric patients with congenital purpura fulminans due to homozygous protein C deficiency, but the use of anticoagulation plus protein C replacement is recommended to reduce the risk of further thrombosis and improve outcomes.
From the Research
Treatment for Late-Stage Purpura Fulminans (PF)
The treatment for late-stage Purpura Fulminans (PF) involves several approaches, including:
- Repletion of natural circulating anticoagulants 2
- Use of therapeutic anticoagulation 2, 3
- Optimization of transfusion support 2, 3
- Treatment of the underlying infection 3, 4
- Measures to improve circulation, infection control, and healing of necrotic tissue 5
- Surgical consultation and potential skin- and soft-tissue-releasing incisions to reduce the incidence of extremity necrosis 5
- Amputation may be necessary in some cases, and should be conservative but may require revision 5
Specific Interventions
Some specific interventions that may be recommended for the treatment of late-stage PF include:
- Corticosteroid therapy to repress the inflammatory process, as seen in a case of PF associated with leukocytoclastic vasculitis triggered by antibiotic intake 6
- Antibiotic treatment, which may be crucial for the outcomes of PF associated with bacterial infections 4
- Supportive care, such as pain management and wound care, to improve patient outcomes and reduce the risk of further complications 5