Long-term remission induced by low-dose rituximab for relapsed and refractory thrombotic thrombocytopenic purpura: A report of two cases

Tong,Hong‑Yan; Ye,Li; Ye,Xing‑Nong; Lu,De‑Min; Li,Ying

doi:10.3892/etm.2015.2797

Long-term remission induced by low-dose rituximab for relapsed and refractory thrombotic thrombocytopenic purpura: A report of two cases

Authors:
- Hong‑Yan Tong
- Li Ye
- Xing‑Nong Ye
- De‑Min Lu
- Ying Li
View Affiliations

Affiliations: Department of Hematology, The First Affiliated Hospital of Zhejiang University, Hangzhou, Zhejiang 310003, P.R. China
Published online on: October 14, 2015 https://doi.org/10.3892/etm.2015.2797
Pages: 2295-2298

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )

Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )

Cited By (CrossRef): 0 citations Loading Articles...

Abstract

Thrombotic thrombocytopenic purpura (TTP) is acquired in the majority of cases. Traditional therapy consists of plasma exchange (PEX), as well as the administration of certain immunosuppressive agents including steroids. A standard dose of rituximab (RTX) at 375 mg/m2 weekly for 4 consecutive weeks was recently demonstrated to have significant activity in patients with acquired TTP. To date, clinicians have limited experience using low‑dose RTX. In the present study, 2 patients were treated with low‑dose RTX at 100 mg weekly for 4 consecutive weeks as a salvage therapy following failure to respond to PEX and other immunosuppressive agents. Prior to RTX therapy, the patients had severely deficient ADAMTS13 activity and detectable anti‑ADAMTS13 inhibitors. The patients achieved complete remission and presented long‑term stabilization during follow‑up. Repeated detection during follow‑up demonstrated that the patients had 100% ADAMTS13 activity and undetectable anti‑ADAMTS13 antibodies. Although further investigation in a prospective clinical trial is required, the use of low‑dose RTX seems to be as effective as a standard dose for patients with relapsing or refractory acquired TTP.

View Figures

View References

1	Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC and Spasoff RA: Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 325:393–397. 1991. View Article : Google Scholar : PubMed/NCBI
2	Tsai HM: Current concepts in thrombotic thrombocytopenic purpura. Annu Rev Med. 57:419–436. 2006. View Article : Google Scholar : PubMed/NCBI
3	Allford SL, Hunt BJ, Rose P and Machin SJ: Haemostasis and Thrombosis Task Force, British Committee for Standards in Haemotology: Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol. 120:556–573. 2003. View Article : Google Scholar : PubMed/NCBI
4	Zaja F, Baccarani M, Mazza P, Bocchia M, Gugliotta L, Zaccaria A, Vianelli N, Defina M, Tieghi A, Amadori S, et al: Dexamethasone plus rituximab yields higher sustained response rates than dexamethasone monotherapy in adults with primary immune thrombocytopenia. Blood. 115:2755–2762. 2010. View Article : Google Scholar : PubMed/NCBI
5	Patel VL, Mahévas M, Lee SY, Stasi R, Cunningham-Rundles S, Godeau B, Kanter J, Neufeld E, Taube T, Ramenghi U, et al: Outcomes 5 years after response to rituximab therapy in children and adults with immune thrombocytopenia. Blood. 119:5989–5995. 2012. View Article : Google Scholar : PubMed/NCBI
6	Zaja F, Vianelli N, Volpetti S, Battista ML, Defina M, Palmieri S, Bocchia M, Medeot M, De Luca S, Ferrara F, et al: Low-dose rituximab in adult patients with primary immune thrombocytopenia. Eur J Haematol. 85:329–334. 2010. View Article : Google Scholar : PubMed/NCBI
7	Zecca M, De Stephano P, Nobili B and Locatelli F: Anti-CD20 monoclonal antibody for the treatment of severe, immune-mediated, pure red cell aplasia and hemolytic anaemia. Blood. 97:3995–39972001. View Article : Google Scholar
8	Fakhouri F, Vernant JP, Veyradier A, Wolf M, Kaplanski G, Binaut R, Rieger M, Scheiflinger F, Poullin P, Deroure B, et al: Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: A study of 11 cases. Blood. 106:1932–1937. 2005. View Article : Google Scholar : PubMed/NCBI
9	Scully M, McDonald V, Cavenagh J, Hunt BJ, Longair I, Cohen H and Machin SJ: A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 118:1746–1753. 2011. View Article : Google Scholar : PubMed/NCBI
10	Froissart A, Buffet M, Veyradier A, Poullin P, Provôt F, Malot S, Schwarzinger M, Galicier L, Vanhille P, Vernant JP, et al: Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French thrombotic microangiopathies reference center. Crit Care Med. 40:104–111. 2012. View Article : Google Scholar : PubMed/NCBI
11	Iioka F, Shimomura D, Ishii T, Maesako Y, Ohgoe K, Nakamura F, Matsuo S and Ohno H: Short- and long-term effects of rituximab for the treatment of thrombotic thrombocytopenic purpura: Four case reports. Int J Hematol. 96:506–512. 2012. View Article : Google Scholar : PubMed/NCBI
12	de la Rubia JD, Moscardó F, Gómez MJ, Guardia R, Rodríguez P, Sebrango A, Zamora C, Debén G, Goterris R, López R, et al: Efficacy and safety of rituximab in adult patients with idiopathic relapsing or refractory thrombotic thrombocytopenic purpura: Results of a Spanish multicenter study. Transfus Apher Sci. 43:299–303. 2010. View Article : Google Scholar : PubMed/NCBI
13	Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B and Machin SJ: British Committee for Standards in Haematology: Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 158:323–335. 2012. View Article : Google Scholar : PubMed/NCBI
14	Pequeño-Luévano M, Villarreal-Martínez L, Jaime-Pérez JC, Gómez-de-León A, Cantú-Rodríguez OG, González-Llano O and Gómez-Almaguer D: Low-dose rituximab for the treatment of acute thrombotic thrombocytopenic purpura: Report of four cases. Hematology. 18:233–236. 2013. View Article : Google Scholar : PubMed/NCBI
15	Taube T, Schmid H, Reinhard H, von Stackelberg A and Overberg US: Effect of a single dose of rituximab in chronic immune thrombocytopenic purpura in childhood. Haematologica. 90:281–283. 2005.PubMed/NCBI
16	Zaja F, Battista ML, Pirrotta MT, Palmieri S, Montagna M, Vianelli N, Marin L, Cavallin M, Bocchia M, Defina M, et al: Lower dose rituximab is active in adults patients with idiopathic thrombocytopenic purpura. Haematologica. 93:930–933. 2008. View Article : Google Scholar : PubMed/NCBI