ADAMTS13 deficiency clinical scoring systems
PLASMIC score and French score
Επιπλέον πληροφορίες
Προειδοποίηση
Αυτό το εργαλείο προορίζεται μόνο για εκπαιδευτικούς σκοπούς, δεν αντιπροσωπεύει και δεν αντικαθιστά επαγγελματικές συμβουλές. Αυτό το εργαλείο δεν πρέπει να χρησιμοποιείται για την παροχή ιατρικών διαγνώσεων ή/και ιατρικών θεραπειών.
General description
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening form of thrombotic microangiopathy, characterized by the deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). PLASMIC and French scores are risk assessment models used to predict the likelihood of severe ADAMTS13 deficiency based on clinical features and relatively simple laboratory tests, with the goal of facilitating urgent plasma replacement therapy.
PLASMIC score is based on the combination of 7 variables: platelet count, combined hemolysis variable, MCV (mean corpuscular volume), INR (international normalized ratio), serum creatinine, the history of cancer in the preceding year and the history of solid-organ or stem cell transplant. The French score is based on 3 variables: platelet count, serum creatinine and positive ANA (antinuclear antibodies). The resulting scores stratify the patients into low, intermediate and high risk of severe ADAMTS13 deficiency.
Both models have been externally validated and are recommended by the International Society on Thrombosis and Haemostasis (ISTH) guidelines for the diagnosis of thrombotic thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening form of thrombotic microangiopathy, characterized by the deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). PLASMIC and French scores are risk assessment models used to predict the likelihood of severe ADAMTS13 deficiency based on clinical features and relatively simple laboratory tests, with the goal of facilitating urgent plasma replacement therapy.
PLASMIC score is based on the combination of 7 variables: platelet count, combined hemolysis variable, MCV (mean corpuscular volume), INR (international normalized ratio), serum creatinine, the history of cancer in the preceding year and the history of solid-organ or stem cell transplant. The French score is based on 3 variables: platelet count, serum creatinine and positive ANA (antinuclear antibodies). The resulting scores stratify the patients into low, intermediate and high risk of severe ADAMTS13 deficiency.
Both models have been externally validated and are recommended by the International Society on Thrombosis and Haemostasis (ISTH) guidelines for the diagnosis of thrombotic thrombocytopenic purpura.
Result ranges: PLASMIC score
Low risk of severe acquired ADAMTS13 deficiency (0–4 %)
0–4Intermediate risk of severe acquired ADAMTS13 deficiency (5–24 %)
5High risk of severe acquired ADAMTS13 deficiency (62–82 %)
6–7Result ranges: French score
Low risk of severe acquired ADAMTS13 deficiency (2 %)
0Intermediate risk of severe acquired ADAMTS13 deficiency (70 %)
1High risk of severe acquired ADAMTS13 deficiency (94 %)
2–3References
Bendapudi, Pavan K., et al. “Derivation and External Validation of the PLASMIC Score for Rapid Assessment of Adults with Thrombotic Microangiopathies: A Cohort Study.” The Lancet Haematology, vol. 4, no. 4, 2017, pp. e157–64. Crossref, https://doi.org/10.1016/s2352-3026(17)30026-1.
Coppo, Paul, et al. “Predictive Features of Severe Acquired ADAMTS13 Deficiency in Idiopathic Thrombotic Microangiopathies: The French TMA Reference Center Experience.” PLoS ONE, edited by Rory Edward Morty, vol. 5, no. 4, 2010, p. e10208. Crossref, https://doi.org/10.1371/journal.pone.0010208.
Zheng, X. Long, et al. “ISTH Guidelines for the Diagnosis of Thrombotic Thrombocytopenic Purpura.” Journal of Thrombosis and Haemostasis, vol. 18, no. 10, 2020, pp. 2486–95. Crossref, https://doi.org/10.1111/jth.15006.
Laurence, Jeffrey et al. “Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis.” Clinical advances in hematology & oncology: H&O, vol. 14, Suppl 11,11, 2016, pp. 2-15.
Ryšavá R. “aHUS a C3 nefropatie: doporučení pro diagnostiku a léčbu” Postgraduální nefrologie, roč. 15, č. 2, 2017, pp. 3-7.
Έκδοση
1
Σχετικά με αυτό το εργαλείο
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening form of thrombotic microangiopathy, characterized by the deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). PLASMIC and French scores are risk assessment models used to predict the likelihood of severe ADAMTS13 deficiency based on clinical features and relatively simple laboratory tests, with the goal of facilitating urgent plasma replacement therapy.
PLASMIC score is based on the combination of 7 variables: platelet count, combined hemolysis variable, MCV (mean corpuscular volume), INR (international normalized ratio), serum creatinine, the history of cancer in the preceding year and the history of solid-organ or stem cell transplant. The French score is based on 3 variables: platelet count, serum creatinine and positive ANA (antinuclear antibodies). The resulting scores stratify the patients into low, intermediate and high risk of severe ADAMTS13 deficiency.
Both models have been externally validated and are recommended by the International Society on Thrombosis and Haemostasis (ISTH) guidelines for the diagnosis of thrombotic thrombocytopenic purpura.
PLASMIC score is based on the combination of 7 variables: platelet count, combined hemolysis variable, MCV (mean corpuscular volume), INR (international normalized ratio), serum creatinine, the history of cancer in the preceding year and the history of solid-organ or stem cell transplant. The French score is based on 3 variables: platelet count, serum creatinine and positive ANA (antinuclear antibodies). The resulting scores stratify the patients into low, intermediate and high risk of severe ADAMTS13 deficiency.
Both models have been externally validated and are recommended by the International Society on Thrombosis and Haemostasis (ISTH) guidelines for the diagnosis of thrombotic thrombocytopenic purpura.
