From the Research
Fibrinogen levels in patients with Thrombotic Thrombocytopenic Purpura (TTP) are typically normal or only slightly decreased, ranging from 150-400 mg/dL, which falls within the normal reference range for most laboratories. This is an important distinguishing feature that helps differentiate TTP from disseminated intravascular coagulation (DIC), where fibrinogen levels are often significantly reduced 1. The preservation of relatively normal fibrinogen levels in TTP occurs because the primary pathophysiology involves platelet aggregation due to unusually large von Willebrand factor multimers resulting from ADAMTS13 deficiency, rather than consumption of clotting factors.
Some key points to consider in the management of TTP include:
- The use of plasma exchange with fresh frozen plasma replacement and immunosuppression with corticosteroids as first-line therapy 2
- The potential addition of anti-VWF therapy with caplacizumab to the front-line setting 1
- The importance of long-term follow-up to monitor for relapse and to diagnose and manage chronic sequelae of the disease 2
- The development of novel therapies, such as recombinant ADAMTS13 products and gene therapy, which show promise for future clinical use 1
Monitoring fibrinogen levels may still be important during the management of TTP, particularly to rule out other concurrent conditions that might affect coagulation. However, the primary focus of treatment remains on addressing the underlying ADAMTS13 deficiency and managing the clinical manifestations of the disease.