Thrombotic thrombocytopenic purpura (TTP) treatment and management
TTP is a rare form of thrombotic microangiopathy (TMA) and is a medical emergency with a mortality rate as high as 90% if left untreated. Even delayed treatment can lead to significant morbidity and mortality.1,2
For suspected cases of acquired thrombotic thrombocytopenic purpura (aTTP), treatment decisions should be made based on clinical suspicion before confirmatory ADAMTS13 results are available.3
Diagnosing and treating TTP can be complicated due to the rarity of the condition and the similarity of its presentation to other TMAs.4
Treating aTTP
While treating an acute episode is the most important, it's also important to ensure you have a long-term management protocol in place.
Acquired TTP (aTTP/iTTP*) treatment
Acquired or immune-mediated TTP (aTTP/iTTP) is the most common form of TTP, representing 90-95% of TTP cases.2
Current treatment methods for aTTP include daily plasma exchange, immunosuppressive therapy, and specific treatments that block disease-driven platelet adhesion.4
*aTTP is also known as iTTP. The terms can be used interchangeably.
Long-term follow-up for aTTP management
Modern therapy for acute aTTP has significantly improved outcomes, with over 90% survival rates. However, the risk of relapse remains significant, particularly within the first year after diagnosis.1,5
After remission, regular monitoring of ADAMTS13 activity, a chemistry panel, complete blood count, and measurement of lactate dehydrogenase (LDH) level is essential.3
Platelet count normalization is not an indication for early treatment termination, as this can put patients at risk for exacerbations.6
If treatment is discontinued prematurely, patients may often see a decline in their platelet count.
Acute intervention and consistent monitoring are key to long-term TTP management
ADAMTS13=a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13.
References: 1. Westwood JP, Scully M. Management of acquired, immune thrombocytopenic purpura (iTTP): beyond the acute phase. Ther Adv Hematol. 2022;13:20406207221112217. doi:10.1177/20406207221112217 eCollection 2022. 2. Soto-Mora JA, Gómez-Espitia LM, Lasalvia P, Castellanos Moreno CA, Casallas Vanegas CA, Londoño Gutiérrez SA. Effectiveness and safety of caplacizumab in acquired thrombotic thrombocytopenic purpura: health technology assessment and classification according to the methodology established in Colombia. Int J Technol Assess Health Care. 2023;39(1):e48. doi:10.1017/S0266462323000442 3. Sukumar S, Lämmle B, Cataland SR. Thrombotic thrombocytopenic purpura: pathophysiology, diagnosis, and management. J Clin Med. 2021;10(3):536. doi:10.3390/jcm10030536 4. Zheng XL, Vesely SK, Cataland SR, et al. ISTH Guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):2486-2495. doi:10.1111/jth.15006 5. Chaturvedi S, Abbas H, McCrae KR. Increased morbidity during long-term follow-up of survivors of thrombotic thrombocytopenic purpura. Am J Hematol. 2015;90(10):E208. doi:10.1002/ajh.24138 Epub 2015 Sep 10. 6. Cuker A, Cataland SR, Coppo P, et al; International Working Group for Thrombotic Thrombocytopenic Purpura. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021;137(14):1855-1861. doi:10.1182/blood.2020009150