Frequency of thrombotic thrombocytopenic purpura diagnosed on the basis of ADMTS13 assay in patients with thrombotic microangiopathy
Paper Details
Frequency of thrombotic thrombocytopenic purpura diagnosed on the basis of ADMTS13 assay in patients with thrombotic microangiopathy
Abstract
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.
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Fauzia Khan1, Saqib Qayyum Ahmad (2020), Frequency of thrombotic thrombocytopenic purpura diagnosed on the basis of ADMTS13 assay in patients with thrombotic microangiopathy; IJB, V17, N6, December, P491-496
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