Welcome to International Network for Natural Sciences | INNSpub

Frequency of thrombotic thrombocytopenic purpura diagnosed on the basis of ADMTS13 assay in patients with thrombotic microangiopathy

Research Paper | December 1, 2020

| Download 8

Fauzia Khan1, Saqib Qayyum Ahmad

Key Words:

Int. J. Biosci.17( 6), 491-496, December 2020

DOI: http://dx.doi.org/10.12692/ijb/17.6.491-496


IJB 2020 [Generate Certificate]


Thrombotic thrombocytopenic purpura (TTP) is a rare disease with an incidence of less than 10 per million per year in the West. In our hospital, clinical diagnosis of TTP is based on detection of thrombotic microangiopathy (TMA) without any evidence of disseminated intravascular coagulation, history of preceding diarrhoeal illness and acute renal failure. Assay of the enzyme ADAMTS13 (a disintegrin and metalloprotease with thrombo- spondin- type 1 motif, member-13), has been described as a sensitive method to diagnose TTP. We carried out a study to see the frequency of TTP in patients with TMA employing ELISA based ADAMTS13 assay. The study was conducted at haematology department, Army Medical College, in collaboration with Military Hospital, Rawalpindi from November 2014 to April 2016. ADAMTS13 assay was carried on serum samples of all the patients by quantitative sandwich enzyme immunoassay. Frequency and percentage of patients showing normal, reduced and severely reduced levels (levels < 10% of the mean of the normal reference range) of ADAMTS13 were calculated. Only one (3.3%) of 30 patients included in the study had severe deficiency of ADAMTS13, falling within the diagnostic range of TTP. Five (16.6%) patients had ADAMTS13 within normal range while 24 (80%) patients had moderately reduced levels of ADAMTS13. TTP, if diagnosed on the basis of ADAMTS13 assay, is a rarer cause of TMA as compared to clinically diagnosed TTP.


Copyright © 2020
By Authors and International Network for
Natural Sciences (INNSPUB)
This article is published under the terms of the Creative
Commons Attribution Liscense 4.0

Frequency of thrombotic thrombocytopenic purpura diagnosed on the basis of ADMTS13 assay in patients with thrombotic microangiopathy

Arnold DM, Patriquin CJ, Nazy I. 2017. Thrombotic microangiopathies: a general approach to diagnosis and management. Canadian Medical Association Journal 189, E153-9.

Barrows BD, Teruya J. 2014. Archives of Pathology & Laboratory Medicine 138, 546-549.

Bianchi V, Robles R, Alberio L, Furlan M, Lämmle B. 2002. Von Willebrand factor–cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood 100(2), 710-713.

Joly BS, Coppo P, Veyradier A. 2017. Thrombotic Thrombocytopenic purpura. Blood 129, 2836-2846.

Kremer Hovinga JA, Coppo P, Lämmle B, Moake JL, Miyata T, Vanhoorelbeke K. 2017. Thrombotic thrombocytopenic purpura. Nature Reviews Disease Primers 3, 17020.

Mannucci PM, Peyvandi F, Palla R. 2016. Thrombotic thombocytopenic purpura and haemolytic uraemic syndrome (congenital and acquired). In: Hoffbrand AV, Higgs DR, Keeling DM, and Mehta AB, Eds. Postgraduate Haematology, 7th ed. UK: Wiley-Blackwell, p 783-794.

Mariotte E, Azoulay E, Galicier L, Rondeau E, Zouiti F, Boisseau P, Poullin P, de Maistre E, Provôt F, Delmas Y, Perez P, Benhamou Y, Stepanian A, Coppo P, Veyradier A, French Reference Center for Thrombotic Microangiopathies. 2016. Epidemiology and pathophysiology of adulthood‐onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross‐sectional analysis of the French national registry for thrombotic microangiopathy. Lancet Haematology 3, e237-45.

Masias C, Cataland SR. 2018. The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis. Blood 132(9), 903-910.

Peigne V, Azoulay E, Coquet I, Mariotte E, Darmon M, Legendre P, Adoui N, Marfaing- Koka A, Wolf M, Schlemmer B, Veyradier A. 2013. The prognostic value of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency in septic shock patients involves interleukin-6 and is not dependent on disseminated intravascular coagulation. Critical Care 17(6), R273.

Pirrotta MT, Bucalossi A, Forconi F, Bocchia M, Mazzotta S, Sammassimo S, Gozzetti A, Lauria F. 2005. Thrombotic thrombocytopenic purpura secondary to an occult adenocarcinoma. The Oncologist 10(4), 299-300.

Systems RD. 2019. Human ADAMTS13 Immunoassay, Quantikine® ELISA. Catalog Number DADT130. https://resources.rndsystems.com/pdfs/datasheets/dadt130.pdf

Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ. 2012. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. British Journal of Haematology 158(3), 323-35.

Zheng XL. 2015. ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura. Annual Review of Medicine 66, 211-225.

Zini G, d’Onofrio G, Briggs C, Erber W, Jou JM, Lee SH, Mc Fadden S, Vives-Corrons JL, Yutaka N, Lesesve JF. 2012. ICSH recommendations for identification, diagnostic value, and quantitation of schistocytes. International Journal of Laboratory Hematology 34(2), 107-16.