Imbalance of the von Willebrand Factor - ADAMTS-13 axis in patients with retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S).
Max BrauneMoritz MetelmannJonathan de FalloisChristian PfrepperAlonso Barrantes-FreerGrit Gesine Ruth HillerSusette UngerEvelyn SeelowJan HalbritterJohann Otto PelzPublished in: Neurological research and practice (2024)
These findings point to an imbalance of the vWF - ADAMTS-13 axis in patients with RVCL-S, that may finally lead to an accumulation of vWF-Ag in renal and cerebral small vessels. Elevated vWF-Ag levels may serve as an early serum marker reflecting disease activity. If confirmed, therapeutic approaches might aim at an inhibition of vWF-Ag or increase of ADAMTS-13 activity in the future.
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