AI Article Synopsis

  • Thrombotic microangiopathy (TMA) is a complex condition involving microvascular thrombosis and can have various causes, with ADAMTS13 deficiency being significant for some patients.
  • Among TMA patients with normal ADAMTS13 activity, treatment options are unclear, and differential diagnoses can include sepsis and autoimmune disorders.
  • A study found that while severe ADAMTS13-deficient patients didn't show elevated levels of procalcitonin (PCT), a significant percentage of ADAMTS13-normal patients tested positive for PCT, suggesting a potential link to sepsis that could aid in timely treatment decisions.

Article Abstract

Thrombotic microangiopathy (TMA) comprises a group of microvascular thrombosis syndromes associated with multiple pathogenic factors. Deficient activity of ADAMTS13 is a pathogenic factor in a subset of TMA patients that provides a strong rationale for plasma exchange treatment. However, the subset of TMA patients with normal ADAMTS13 activity remains a heterogeneous group of patients in which the appropriate treatment is not well understood. In addition to the common forms of TMA thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome, the differential diagnosis of TMA may include sepsis, autoimmune disorders, and disseminated intravascular coagulation. Optimal treatment of TMA depends on timely recognition of treatable pathogenic factors. We hypothesized that sepsis is a rapidly identifiable pathogenic factor in a subset of TMA patients. To test this hypothesis, we retrospectively measured the rapid biomarkers of sepsis C-reactive protein (CRP) and procalcitonin (PCT), in a repository of pretreatment plasma samples from 61 TMA patients treated with plasma exchange. Levels were analyzed in 31 severely ADAMTS13-deficient and 30 ADAMTS13-normal patients. None of the 31 patients with severe deficiency of ADAMTS13 had elevated PCT. However, 11 of 30 (37%) non-ADAMTS13-deficient patient samples were strongly positive for PCT. These patient samples also had a >10-fold higher median CRP level than patients with normal PCT. We conclude that rapid assays may help identify sepsis in a subset of TMA patients.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2750870PMC
http://dx.doi.org/10.1002/jca.20205DOI Listing

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