Changes in plasma von Willebrand factor and von Willebrand factor cleaving protease in thrombotic thrombocytopenic purpura: A case report

Gao,Yu; Wang,Liping; Nan,Guangxian

doi:10.3892/etm.2017.5361

Changes in plasma von Willebrand factor and von Willebrand factor cleaving protease in thrombotic thrombocytopenic purpura: A case report

Authors:
- Yu Gao
- Liping Wang
- Guangxian Nan
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Affiliations: Second Department of Neurology, China‑Japan Union Hospital of Jilin University, Changchun, Jilin 130033, P.R. China
Published online on: October 24, 2017 https://doi.org/10.3892/etm.2017.5361
Pages: 205-209
Copyright: © Gao et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

The present case report described a patient with thrombotic thrombocytopenic purpura (TTP), who presented to the China‑Japan Union Hospital of Jilin University (Changchun, China), and the changes in plasma von Willebrand factor (vWF) and vWF cleaving protease (vWF‑cp) observed during treatment. Computed tomography and diffusion‑weighted magnetic resonance (MR) imaging of the brain, cerebral MR angiography and other experimental tests were performed and the patient was subsequently diagnosed with TTP. The patient underwent treatment with plasma exchange, glucocorticoids and supportive care. Hematologic and biochemical parameters began to gradually improve over the 12 days after admission; platelet count, serum creatinine and serum lactate dehydrogenase concentrations returned to their normal ranges, plasma vWF concentration decreased to normal levels over the 30 days after admission, and vWF‑cp activity increased compared with the levels detected in healthy volunteers. Monthly rituximab treatment was administered 4 times following patient discharge to prevent relapse, and no recurrence was detected at the 20‑month follow‑up. Plasma exchange therapy is effective in patients with TTP. After low‑dose rituximab treatment, recurrent TTP has not been detected found till now. In the present case, vWF concentration and vWF‑cp activity were measured at 8, 10, 23 and 32 days after admission; compared with the control group, patient's vWF concentration gradually decreased and vWF‑cp activity slowly increased, suggesting that the patient had a favorable prognosis and a low risk of recurrence.

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1	Amorosi EL and Ultmann JE: Thrombotic thrombocytopenic purpura: Report of 16 cases and review of the literature. Medicine. 45:139–159. 1966. View Article : Google Scholar
2	Allford SL, Hunt BJ, Rose P and Machin SJ: Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology: 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
3	Said A, Haddad RY, Stein R and Lerma EV: Thrombotic thrombocytopenic purpura. Dis Mon. 60:500–504. 2014. View Article : Google Scholar : PubMed/NCBI
4	Edel E, Al-Ali HK, Seeger S, Kauschat D and Matthes G: efficacy and safety profile of solvent/detergent plasma in the treatment of acute thrombotic thrombocytopenic purpura: A single-center experience. Transfus Med Hemother. 37:13–19. 2010. View Article : Google Scholar : PubMed/NCBI
5	Ye YF, Wang YS and Shen ZY: National Guide to Clinical Laboratory Procedures. Third. Southeast University Press; Nanjing: 2006
6	Rovner BW and Folstein MF: Mini-mental state exam in clinical practice. Hosp Pract (Off Ed). 22:991031061101987.PubMed/NCBI
7	Chalmers J: The 1999 WHO-ISH guidelines for the management of hypertension. Med J Aust. 171:458–459. 1999.PubMed/NCBI
8	Bell WR, Braine HG, Ness PM and Kickler TS: Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med. 325:398–403. 1991. View Article : Google Scholar : PubMed/NCBI
9	George JN: How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood. 116:4060–4069. 2010. View Article : Google Scholar : PubMed/NCBI
10	Bukowski RM, Hewlett JS, Harris JW, Hoffman GC, Battle JD Jr, Silverblatt E and Yang IY: Exchange transfusions in the treatment of thrombotic thrombocytopenic purpura. Semin Hematol. 13:219–232. 1976.PubMed/NCBI
11	Fontana S, Hovinga JA Kremer, Lämmle B and Taleghani B Mansouri: Treatment of thrombotic thrombocytopenic purpura. Vox Sang. 90:245–254. 2006. View Article : Google Scholar : PubMed/NCBI
12	Rizzo C, Rizzo S, Scirè E, Di Bona D, Ingrassia C, Franco G, Bono R, Quintini G and Caruso C: Thrombotic thrombocytopenic purpura: A review of the literature in the light of our experience with plasma exchange. Blood Transfus. 10:521–532. 2012.PubMed/NCBI
13	Westwood JP, Webster H, McGuckin S, McDonald V, Machin SJ and Scully M: Rituximab for thrombotic thrombocytopenic purpura: Benefit of early administration during acute episodes and use of prophylaxis to prevent relapse. J Thromb Haemost. 11:481–490. 2013. View Article : Google Scholar : PubMed/NCBI
14	Heidel F, Lipka DB, von Auer C, Huber C, Scharrer I and Hess G: Addition of rituximab to standard therapy improves response rate and progression-free survival in relapsed or refractory thrombotic thrombocytopenic purpura and autoimmune haemolytic anaemia. Thromb Haemost. 97:228–233. 2007.PubMed/NCBI
15	Foley SR, Webert K, Arnold DM, Rock GA, Clark WF, Barth D and Sutton DM; Members of the Canada Apheresis Group (CAG), : A Canadian phase II study evaluating the efficacy of rituximab in the management of patients with relapsed/refractory thrombotic thrombocytopenic purpura. Kidney Int Suppl. S55–S58. 2009. View Article : Google Scholar : PubMed/NCBI
16	Tun NM and Villani GM: Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: A systematic review with pooled data analysis. J Thromb Thrombolysis. 34:347–359. 2012. View Article : Google Scholar : PubMed/NCBI
17	Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL and Deykin D: Unusually large plasma factor VIII: Von willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med. 307:1432–1435. 1982. View Article : Google Scholar : PubMed/NCBI
18	Furlan M, Robles R and Lämmle B: Partial purification and characterization of a protease from human plasma cleaving von willebrand factor to fragments produced by in vivo proteolysis. Blood. 87:4223–4234. 1996.PubMed/NCBI
19	Gerritsen HE, Robles R, Lämmle B and Furlan M: Partial amino acid sequence of purified von willebrand factor-cleaving protease. Blood. 98:1654–1661. 2001. View Article : Google Scholar : PubMed/NCBI
20	Hovinga JA Kremer and Lämmle B: Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program. 2012:610–616. 2012.PubMed/NCBI
21	Duraković N, Radonić R and Gasparović V: Thrombotic thrombocytopenic purpura-the role of ADAMTS13 assay in clinical practice. Coll Antropol. 34:1087–1091. 2010.PubMed/NCBI
22	Hovinga JA Kremer, Vesely SK, Terrell DR, Lämmle B and George JN: Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 115:1500–1511. 2010. View Article : Google Scholar : PubMed/NCBI
23	Peyvandi F, Lavoretano S, Palla R, Feys HB, Vanhoorelbeke K, Battaglioli T, Valsecchi C, Canciani MT, Fabris F, Zver S, et al: ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica. 93:232–239. 2008. View Article : Google Scholar : PubMed/NCBI
24	Arya M, Anvari B, Romo GM, Cruz MA, Dong JF, McIntire LV, Moake JL and López JA: Ultralarge multimers of von willebrand factor form spontaneous high-strength bonds with the platelet glycoprotein Ib-IX complex: Studies using optical tweezers. Blood. 99:3971–3977. 2002. View Article : Google Scholar : PubMed/NCBI