Early administration of caplacizumab combined with plasma exchange for thrombotic microangiopathy due to malignant hypertension: a case report

Maisons V, Duval A, Mesnard L, Frimat M, Fakhouri F, Grangé S, et al. Assessment of epidemiology and outcomes of adult patients with kidney-limited thrombotic microangiopathies. Kidney Int. 2024;105(5):1100–12.

Article  PubMed  CAS  Google Scholar 

George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654–66.

Article  PubMed  CAS  Google Scholar 

Scully M, Cataland S, Coppo P, de la Rubia J, Friedman KD, Kremer Hovinga J, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017;15(2):312–22.

Article  PubMed  CAS  Google Scholar 

Fakhouri F, Zuber J, Frémeaux-Bacchi V, Loirat C. Haemolytic uraemic syndrome. Lancet. 2017;390(10095):681–96.

Article  PubMed  Google Scholar 

Kremer Hovinga JA, Coppo P, Lämmle B, Moake JL, Miyata T, Vanhoorelbeke K. Thrombotic thrombocytopenic purpura. Nat Rev Dis Primers. 2017;3:17020.

Article  PubMed  Google Scholar 

Werion A, Storms P, Zizi Y, Beguin C, Bernards J, Cambier JF, et al. Epidemiology, outcomes, and complement gene variants in secondary thrombotic microangiopathies. Clin J Am Soc Nephrol. 2023;18(7):881–91.

Article  PubMed  PubMed Central  Google Scholar 

Schofield J, Hosseinzadeh S, Burton K, Pavord S, Dutt T, Doree C, et al. Drug-induced thrombotic thrombocytopenic purpura: a systematic review and review of European and North American pharmacovigilance data. Br J Haematol. 2023;201(4):766–73.

Article  PubMed  CAS  Google Scholar 

Schimmer RR, Sutter T, Bachofner A, Ranieri E, Rodewald AK, Kremer Hovinga JA, et al. Successful management of refractory immune-mediated thrombotic thrombocytopenic purpura during pregnancy and delivery using the anti-VWF nanobody caplacizumab. Br J Haematol. 2024;204(5):1994–8.

Article  PubMed  CAS  Google Scholar 

Genest DS, Patriquin CJ, Licht C, John R, Reich HN. Renal thrombotic microangiopathy: a review. Am J Kidney Dis. 2023;81(5):591–605.

Article  PubMed  CAS  Google Scholar 

Moake JL. Thrombotic thrombocytopenic purpura: the systemic clumping “plague.” Annu Rev Med. 2002;53:75–88.

Article  PubMed  CAS  Google Scholar 

Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998;339(22):1578–84.

Article  PubMed  CAS  Google Scholar 

Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339(22):1585–94.

Article  PubMed  PubMed Central  CAS  Google Scholar 

George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(19):1847–8.

PubMed  Google Scholar 

Cavero T, Auñón P, Caravaca-Fontán F, Trujillo H, Arjona E, Morales E, et al. Thrombotic microangiopathy in patients with malignant hypertension. Nephrol Dial Transplant. 2023;38(5):1217–26.

Article  PubMed  CAS  Google Scholar 

He F, Zhou Z, Zhao S, Li W, Lian X, Yu J, et al. Impact of Overweight on Renal Prognosis in Malignant Hypertension Patients With Thrombotic Microangiopathy. J Clin Lab Anal. 2024:e25118.

Iwamura N, Matsukuma Y, Katafuchi E, Nakano Y, Tsutsumi K, Ueno Y, et al. Managing malignant hypertension with renal TMA: a case for caution in blood-pressure reduction. CEN Case Rep. 2024. https://doi.org/10.1007/s13730-024-00933-8.

Article  PubMed  PubMed Central  Google Scholar 

van den Born BJ, Koopmans RP, Groeneveld JO, van Montfrans GA. Ethnic disparities in the incidence, presentation and complications of malignant hypertension. J Hypertens. 2006;24(11):2299–304.

Article  PubMed  Google Scholar 

Akimoto T, Muto S, Ito C, Takahashi H, Takeda S, Ando Y, et al. Clinical features of malignant hypertension with thrombotic microangiopathy. Clin Exp Hypertens. 2011;33(2):77–83.

Article  PubMed  CAS  Google Scholar 

Zhang B, Xing C, Yu X, Sun B, Zhao X, Qian J. Renal thrombotic microangiopathies induced by severe hypertension. Hypertens Res. 2008;31(3):479–83.

Article  PubMed  Google Scholar 

van den Born BJ, Honnebier UP, Koopmans RP, van Montfrans GA. Microangiopathic hemolysis and renal failure in malignant hypertension. Hypertension. 2005;45(2):246–51.

Article  PubMed  Google Scholar 

van den Born BJ, van der Hoeven NV, Groot E, Lenting PJ, Meijers JC, Levi M, et al. Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension. Hypertension. 2008;51(4):862–6.

Article  PubMed  Google Scholar 

Rubin S, Cremer A, Boulestreau R, Rigothier C, Kuntz S, Gosse P. Malignant hypertension: diagnosis, treatment and prognosis with experience from the Bordeaux cohort. J Hypertens. 2019;37(2):316–24.

Article  PubMed  CAS  Google Scholar 

Peyvandi F, Scully M, Kremer Hovinga JA, Cataland S, Knöbl P, Wu H, et al. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2016;374(6):511–22.

Article  PubMed  CAS  Google Scholar 

Scully M, Cataland SR, Peyvandi F, Coppo P, Knöbl P, Kremer Hovinga JA, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019;380(4):335–46.

Article  PubMed  CAS  Google Scholar 

Matsumoto M. Novel treatment strategies for acquired thrombotic thrombocytopenic purpura. Rinsho Ketsueki. 2023;64(9):1124–30.

PubMed  Google Scholar 

Matsumoto M, Miyakawa Y, Kokame K, Ueda Y, Wada H, Higasa S, et al. Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) in Japan 2023. Int J Hematol. 2023;118(5):529–46.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Sánchez-Corral P, González-Rubio C, Rodríguez de Córdoba S, López-Trascasa M. Functional analysis in serum from atypical Hemolytic Uremic Syndrome patients reveals impaired protection of host cells associated with mutations in factor H. Mol Immunol. 2004;41(1):81–4.

Yoshida Y, Miyata T, Matsumoto M, Shirotani-Ikejima H, Uchida Y, Ohyama Y, et al. A novel quantitative hemolytic assay coupled with restriction fragment length polymorphisms analysis enabled early diagnosis of atypical hemolytic uremic syndrome and identified unique predisposing mutations in Japan. PLoS ONE. 2015;10(5): e0124655.

Article  PubMed  PubMed Central  Google Scholar 

Coppo P, Schwarzinger M, Buffet M, Wynckel A, Clabault K, Presne C, et al. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS ONE. 2010;5(4): e10208.

Article  PubMed  PubMed Central  Google Scholar 

Bendapudi PK, Hurwitz S, Fry A, Marques MB, Waldo SW, Li A, et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol. 2017;4(4):e157–64.

Article  PubMed  Google Scholar 

Grall M, Azoulay E, Galicier L, Provôt F, Wynckel A, Poullin P, et al. Thrombotic thrombocytopenic purpura misdiagnosed as autoimmune cytopenia: causes of diagnostic errors and consequence on outcome. Experience of the French thrombotic microangiopathies reference centre. Am J Hematol. 2017;92(4):381–7.

Lesesve JF, Salignac S, Lecompte T. Laboratory measurement of schistocytes. Int J Lab Hematol. 2007;29(2):149–51.

Article  PubMed  Google Scholar 

Sethi SK, S S, Nair A, Soni K, Bihari Bansal S, Rana AS, et al. What came first, atypical hemolytic uremic syndrome or malignant hypertension: a clinical dilemma. Ren Fail. 2024;46(1):2327484.

Coppo P, Bubenheim M, Azoulay E, Galicier L, Malot S, Bigé N, et al. A regimen with caplacizumab, immunosuppression, and plasma exchange prevents unfavorable outcomes in immune-mediated TTP. Blood. 2021;137(6):733–42.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Mandyam S, Fatmi SS, Banzon G, Kaur P, Katamreddy Y, Parghi D, et al. A rare case of severe manifestation of COVID-19 infection presenting as immune-related thrombotic thrombocytopenic purpura with multiorgan involvement treated with plasmapheresis, steroids, rituximab, and caplacizumab. Cureus. 2022;14(7): e26961.

PubMed  PubMed Central 

Comments (0)

No login
gif