Validation of the PLASMIC score for predicting ADAMTS13 activity &lt;10% in patients with suspected thrombotic thrombocytopenic purpura in Alberta, Canada

Wynick, Chris; Britto, Joseph; Sawler, Daniel; Parker, Arabesque; Karkhaneh, Mohammad; Goodyear, M. Dawn; Sun, Hao

doi:10.1016/j.thromres.2020.09.012

Cited by 20 publications

(22 citation statements)

References 16 publications

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“…Both scoring systems require ruling out the possibility of disseminated malignancy and the history of hematopoitic progenitor transplantation. Several other studies have independently validated the PLASMIC score 51‐54 . Paydary et al performed a meta analysis of 970 cases from 13 eligible studies published and demonstrated that a PLASMIC score ≥5 has a sensitivity and specificity of 0.99 (0.91–1.0, 95% confidence interval [CI]) and 0.57 (0.41–0.72, 95% CI), respectively 53 .…”

Section: Clinical Risk Assessment Scoresmentioning

confidence: 99%

The standard of care for immune thrombotic thrombocytopenic purpura today

Zheng

2021

Journal of Thrombosis and Haemostasis

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Immune thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal blood disorder, primarily caused by acquired deficiency of ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin-1 Domain, member 13), 1,2 a plasma metalloprotease that cleaves von Willebrand factor (VWF). 3,4 VWF is a multimeric adhesive glycoprotein that is synthesized and released from endothelial cells and megakaryocytes/platelets. 5,6 The proteolytic cleavage of endothelial ultra-large VWF (ULVWF) by plasma ADAMTS-13 is essential for normal hemostasis. An inability to cleave the ULVWF anchored on endothelial surface, in circulating blood, and at the sites of vascular injury leads to exaggerated platelet adhesion, agglutination, and formation of occlusive thrombi in small arterioles and capillaries, 7-9 a characteristic feature of iTTP pathology. 10 Without early recognition and prompt management, iTTP is universally fatal. Therapeutic plasma exchange (TPE) has been the standard of care for nearly three decades; it reduces mortality rate to less than 10% to 20%. [11][12][13] Remarkable progresses have been made in recent years in rapid diagnosis and prompt management of acute iTTP, leading to further reduction of the mortality rate.

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Section: Clinical Risk Assessment Scoresmentioning

confidence: 99%

The standard of care for immune thrombotic thrombocytopenic purpura today

Zheng

2021

Journal of Thrombosis and Haemostasis

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“…Bendapudi et al created the PLASMIC score using two cohorts with a high prevalence of TTP (14 to 47%) and demonstrated its accuracy for TTP diagnosis (AUC 0.91-0.96) (10). Further studies with a high TTP prevalence (25 to 70%) confirmed the good diagnostic value of both the PLASMIC and French scores (23)(24)(25)(26)(27)(28)(29). However, they also included highly selected patients recruited among those that had ADAMTS13 measurement (30).…”

Section: Discussionmentioning

confidence: 99%

Proteinuria Increases the PLASMIC and French Scores Performance to Predict Thrombotic Thrombocytopenic Purpura in Patients With Thrombotic Microangiopathy Syndrome

Fage

Orvain

Henry

et al. 2022

Kidney International Reports

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“…Higher percentages of schistocytes on peripheral smear are more characteristic of TTP than of severe PE/HELLP (2% to 5% vs. <1%), also LDH levels of TTP are often >1000 IU, while a much less rise is generally seen in severe PE/HELLP. 9 Moreover, regarding the PLASMIC score, PE with severe features/HELLP usually scores 5 points or less, while a high-risk score (≥6) is a strong predictor of severe ADAMTS-13 deficiency, with a sensitivity of 0.85 (95% confidence interval [CI], 0.67-0.94) and specificity of 0.89 (95% CI, 0.81-0.94). 10 A PLASMIC score of 6 or more accounts for 72% risk of ADAMS-13 deficiency.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura and acquired immunodeficiency syndrome diagnosed in pregnancy: Case report

Marins

Oppermann

2021

J of Obstet and Gynaecol

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Thrombotic thrombocytopenic purpura (TTP) is a medical emergency that demands prompt diagnosis to allow lifesaving treatment: plasmapheresis. TTP during pregnancy is rare, with estimated prevalence of 1/200 000, and even rarer in association with acquired immunodeficiency syndrome, with only two cases reported. Differential diagnosis includes HELLP syndrome (hemolysis elevated liver enzymes and low platelets), sepsis, intravascular‐disseminated coagulation, and acquired autoimmune diseases, each one with its unique treatment and prognosis. A case of a pregnant woman at 26th week with sudden onset of left hand paresthesia and purpura is reported. PLASMIC score showed high risk for ADAMTS‐13 deficiency and diagnosis of TTP was made. Human immunodeficiency virus screening test was positive on admission. Plasmapheresis and antiretroviral therapy were initiated and the delivery of a healthy newborn at full‐term gestation was achieved, unlike other cases in literature. No obstetric complications were observed and the follow‐up shows no signs of disease recurrence.

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Validation of the PLASMIC score for predicting ADAMTS13 activity <10% in patients with suspected thrombotic thrombocytopenic purpura in Alberta, Canada

Cited by 20 publications

References 16 publications

The standard of care for immune thrombotic thrombocytopenic purpura today

The standard of care for immune thrombotic thrombocytopenic purpura today

Proteinuria Increases the PLASMIC and French Scores Performance to Predict Thrombotic Thrombocytopenic Purpura in Patients With Thrombotic Microangiopathy Syndrome

Thrombotic thrombocytopenic purpura and acquired immunodeficiency syndrome diagnosed in pregnancy: Case report

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