What is the treatment for Purpura fulminans (a rare blood disorder) complicated by sepsis (systemic inflammatory response syndrome)?

From the Guidelines

Purpura fulminans complicated by sepsis requires immediate aggressive treatment, including broad-spectrum antibiotics, fluid resuscitation, and correction of coagulation abnormalities, with protein C concentrate administration being a crucial component if available, as recommended by the most recent guidelines 1. The management of purpura fulminans involves several key components:

• Broad-spectrum antibiotics targeting common causative organisms like Neisseria meningitidis, Streptococcus pneumoniae, and Staphylococcus aureus, with initial empiric therapy including a combination of vancomycin and a carbapenem such as meropenem or ceftriaxone, as suggested by the Surviving Sepsis Campaign guidelines 1.
• Fluid resuscitation, typically requiring 20-30 ml/kg of crystalloids in the first hour, followed by vasopressor support if hypotension persists.
• Correction of coagulation abnormalities with fresh frozen plasma, cryoprecipitate to maintain fibrinogen >100 mg/dL, and platelet transfusions to keep counts above 50,000/μL, as recommended by the Surviving Sepsis Campaign guidelines 1.
• Protein C concentrate administration, with a dose of 50-100 IU/kg initially, followed by 50 IU/kg every 6 hours, is crucial in the treatment of purpura fulminans, especially in cases of protein C deficiency, as suggested by the American Society of Hematology guidelines 1. Surgical consultation is necessary for potential debridement of necrotic tissue. The underlying pathophysiology involves microvascular thrombosis, endothelial damage, and consumption of coagulation factors, leading to the characteristic purpuric skin lesions and organ dysfunction. Early recognition and aggressive intervention are crucial for improving survival in this rapidly progressive condition.

From the Research

Treatment Approaches for Purpura Fulminans Complicated by Sepsis

The treatment for Purpura fulminans (PF) complicated by sepsis involves a multifaceted approach, including:

• Repletion of natural circulating anticoagulants, such as protein C, to control microvascular clotting and promote cytoprotective effects 2
• Therapeutic anticoagulation to prevent further thrombosis and tissue damage 2
• Optimization of transfusion support to manage coagulopathy and anemia 2
• Early recognition and intervention for sepsis, including administration of intravenous fluids, antibiotics, and supportive care 3, 4
• Consideration of activated protein C (APC) therapy, although its efficacy and cost-effectiveness require further evaluation 5

Management of Sepsis-Associated Purpura Fulminans

Sepsis-associated PF is a medical emergency that requires prompt diagnosis and treatment, including:

• Aggressive management of sepsis with intravenous fluids, antibiotics, and conventional and nonconventional therapies 4
• Early institution of therapy to prevent significant morbidity and mortality, as delayed treatment can lead to poor outcomes 4
• Consideration of various treatment modalities, such as hyperbaric oxygen therapy and IVIG, although their benefits are still questionable 4

Key Considerations in Treatment

• Prompt recognition of the characteristic skin rash associated with PF is crucial for early diagnosis and treatment 4
• Waiting for a skin biopsy result can delay diagnosis and lead to significant morbidity and mortality 4
• A multidisciplinary approach, involving consultant hematologists and other specialists, is often necessary to manage the complex clinical situation of PF 2