From the Guidelines
The optimal duration of anticoagulation for purpura fulminans is typically lifelong therapy for patients with congenital thrombophilias, while acquired cases may be treated until the precipitating factor resolves, usually within 3-6 months. Initial treatment involves immediate anticoagulation with unfractionated heparin or low molecular weight heparin (such as enoxaparin 1 mg/kg twice daily), followed by transition to warfarin with a target INR of 2-3 or continued LMWH 1. For patients with protein C or S deficiency, replacement therapy with protein C concentrate (50-100 IU/kg initially, then 50 IU/kg every 6-12 hours) or fresh frozen plasma (10-15 mL/kg every 8-12 hours) should be administered alongside anticoagulation, as suggested by the ASH guideline panel 1.
Key Considerations
- Treatment duration should be individualized based on the underlying cause, with congenital thrombophilias often requiring indefinite anticoagulation while acquired cases may be treated until the precipitating factor resolves.
- Regular monitoring of coagulation parameters and clinical response is essential, with gradual tapering considered only after complete resolution of skin lesions and normalization of coagulation markers.
- Liver transplantation is curative of protein C deficiency but has its own acute and chronic risks and burden of care, and the optimal therapy depends on the values and preferences of the family, as well as local health service factors 1.
Treatment Approach
- The approach to treatment should prioritize minimizing morbidity and mortality, while also considering the quality of life for patients with purpura fulminans.
- A multidisciplinary team should be involved in the care of these patients, including hematologists, intensivists, and other specialists as needed.
- Patients with purpura fulminans due to homozygous protein C deficiency should be considered for liver transplantation, anticoagulation, or protein C replacement, depending on the individual case and the values and preferences of the family 1.
From the Research
Anticoagulation Therapy for Purpura Fulminans
The recommended duration of anticoagulation therapy for Purpura Fulminans is not explicitly stated in the provided studies. However, the studies suggest the following:
- The use of anticoagulant therapy, such as heparin and warfarin, in the treatment of Purpura Fulminans 2
- The duration of anticoagulation therapy may vary depending on the individual case, with one study reporting treatment with heparin and warfarin for four days until the INR was more than 3.5 and the d-dimer level was less than 230 ng/mL 2
- Another study reports the use of protein C concentrate to treat severe Purpura Fulminans in a homozygous protein C-deficient infant for 8 months until oral anticoagulation was initiated 3
- The studies also highlight the importance of monitoring treatment with markers such as D-dimer, fibrin monomer, thrombin-antithrombin complex, and prothrombin fragment 1 + 2 3
Key Considerations
- The fundamental pathologic lesion in Purpura Fulminans is a failure of the anticoagulant protein C pathway, which leads to uncontrolled microvascular clotting and inadequate protein C-mediated cytoprotective effects 4
- Anticoagulation therapy must be considered if Purpura Fulminans is present, despite the risk of bleeding 5
- The use of recombinant activated protein C (APC) has been reported to be safe and effective for the treatment of Purpura Fulminans in severe genetic protein C deficiency 2