Elevated plasma levels of syndecan-1 (Sdc-1) and soluble thrombomodulin (sTM) predicted worse outcomes from immune-mediated thrombotic thrombocytopenic purpura (iTTP) in hospitalized patients, according to research in Blood Advances.
ITTP is a blood disorder resulting from acquired deficiency of plasma ADAMTS13 activity. The mortality rate is between 10% and 20%, and clinical parameters to predict outcomes in practice is lacking. The study authors sought to develop a measure to assess risk for a more intense treatment strategy for high-risk patients.
The study included 91 patients with iTTP with 107 acute episodes. Patient outcomes were compared with healthy controls. Patients were followed through outpatient visits and interviews. Of the 107 episodes, 63 (58.9%) were first episodes and 44 (41.1%) were recurrent episodes. A total of 69.2% of patients had comorbidities, including hypertension, diabetes, lupus and HIV infection. The overall mortality rate was 10.3%, with the median time of death being 10 days from admission.
Sdc-1 plasma and sTM levels were higher at admission in patients with acute iTTP compared with healthy controls. Patients with iTTP had median Sdc-1 plasma levels of 1.5 ng/mL and median sTM levels of 4.7 ng/mL at admission before therapeutic plasma exchange. Patients with abnormal Glasgow coma scores tended to have higher Sdc-1 plasma levels and higher sTM levels.
Based on analysis, the authors identified cutoffs of 3.3 ng/mL for Sdc-1 and 4.0 ng/mL for sTM as predictors of probable death.
Some patients saw a decrease in levels during remission; however, patients with an increase in both levels at clinical remission had higher rates of disease recurrence at 3 months, than those who did not have an increase in both levels.
The authors conclude that these results may be used for risk stratification and a molecular basis for therapeutic intervention.
Disclosures: Some authors have declared affiliations with or received grant support from the pharmaceutical industry. Please refer to the original study for a full list of disclosures.
Lu R, Sui J, Zheng XL. Elevated plasma levels of syndecan-1 and soluble thrombomodulin predict adverse outcomes in thrombotic thrombocytopenic purpura. Blood Adv. 2020;4(21):5378-5388. doi:10.1182/bloodadvances.2020003065.