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

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.

A modified PLASMIC score including the lactate dehydrogenase/the upper limit of normal ratio more accurately identifies Chinese thrombotic thrombocytopenic purpura patients than the original PLASMIC score

“…In order to solve this difficulty, many doctors and scientists had tried to use available clinical data to predict TTP, such as the Bentley score, Coppo score, the PLASMIC score, and so on . Among the scores, the PLASMIC score has proved to be practical and effective, which was validated in some studies . However, according to our data, we found that the PLASMIC score may be not suitable to our Chinese suspected TTP patients.…”

Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta‐analysis

“…Twelve articles met eligibility criteria, one of which reported the results of 2 distinct studies, for a total of 13 eligible studies (Figure 1). 5,16‐26 …”

“…One study enrolled patients with disseminated intravascular coagulation as controls 21 ; the other only enrolled patients with confirmed TTP 26 . Risk of bias in the patient selection domain was also classified as high in one study due to inappropriate eligibility criteria because this study excluded patients with cancer 20 …”

Application of PLASMIC score in prediction of ADAMTS13 deficiency in a pediatric case of acquired thrombotic thrombocytopenic purpura

“…Since acquired TTP in pediatric patients is exceedingly rare (with an annual incidence of 0.09 cases per million children year, approximately 3% of the incidence rate among adults), there is little published experience on the application of PLASMIC scoring in this patient population. To our knowledge, only two patients under the age of 21 years old, both of whom had pregnancy‐associated thrombotic microangiopathy, have had their PLASMIC scores published; both had scores of 5 and ADAMTS13 >10% . Here we report a pediatric case of acquired TTP and application of the PLASMIC score in predicting ADAMTS13 deficiency.…”