Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Apr-Jun;41(2):119-124.
doi: 10.1016/j.htct.2018.10.002. Epub 2019 Feb 18.

Plasmic score applicability for the diagnosis of thrombotic microangiopathy associated with ADAMTS13-acquired deficiency in a developing country

Deivide Sousa Oliveira  1 , Tadeu G Lima  2 , Fernanda L Neri Benevides  2 , Suzanna A Tavares Barbosa  3 , Maria A Oliveira  4 , Natália P Boris  4 , Herivaldo F Silva  2
Affiliations

Plasmic score applicability for the diagnosis of thrombotic microangiopathy associated with ADAMTS13-acquired deficiency in a developing country

Deivide Sousa Oliveira et al. Hematol Transfus Cell Ther. 2019 Apr-Jun.

Abstract

Background: Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disease that requires early diagnosis and treatment that can be made possible by applying the PLASMIC score. This study aims to evaluate this score applicability for patients with suspected TTP in a developing country.

Methods: This was a retrospective study performed at a tertiary hospital in the northeastern region of Brazil. Patients were analyzed in two groups: ADAMTS13 activity <10% and activity >10%. Patients were stratified according to the PLASMIC score, and the level of agreement between the PLASMIC score and the ADAMTS13 activity was evaluated.

Results: Eight patients with thrombotic microangiopathy were included. Four patients had ADAMTS13 activity <10%, all with a PLASMIC score =6. The other four had ADAMTS13 activity >10%, all with a score <6. Based on a score =6 for presumptive diagnosis of TTP, we attained a 100% diagnostic accuracy in our sample. The PLASMIC score was also able to accurately predict response to plasma exchange and the risk of long-term unfavorable outcomes.

Conclusions: The reproducibility of the PLASMIC score was quite satisfactory in our sample. It accurately discriminates between patients who had ADAMTS13 deficiency and those with normal enzyme activity, precluding the need for specific laboratory evaluation, which is not always available. This score can be useful for an early diagnosis and indicates which patients will benefit from the treatment in developing countries.

Keywords: ADAMTS13; Plasmic; TTP; aHUS.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Patients’ enrolment in the study.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Moschcowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc N Y Pathol Soc. 1924;24:21–24.
    1. Furlan M., Lämmle B. Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease. Best Pract Res Clin Haematol. 2001;14:437–454. - PubMed
    1. Kremer Hovinga J.A., Coppo P., Bernhard L., Moake J.L., Miyata T., Vanhoorelbeke K. Thrombotic thrombocytopenic purpura. Nat Rev Dis Primers. 2017;3:17020. - PubMed
    1. Kremer Hovinga J.A., Vesely S.K., Terell D.R., Lammle B., George J.N. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010;115:1500–1511. - PubMed
    1. George J.N., Al-Nouri Z.L. Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Hematology Am Soc Hematol Educ Program. 2012;2012:604–609. - PubMed