Prognosis

Patient prognosis is optimal when a prompt diagnosis is made and treatment is started as soon as possible. Neurological or cardiac involvement at presentation, need for intubation and older age are poor prognostic signs.[1] Race also impacts prognosis, non-white people have an increased risk of exacerbation or relapse, but lower mortality.[1]

With the use of plasma exchange, mortality has fallen from >90% to between 10% and 30%. Approximately 90% of patients will respond to plasma exchange within 3 weeks, with most responding within 10 days. Although who will respond cannot be predicted, it appears that those without a severe von Willebrand factor cleaving enzyme (ADAMTS-13) deficiency and without evidence of inhibitors to ADAMTS-13 are more likely to have transient or incomplete responses to therapy.[76] Other laboratory tests (e.g., platelet count) are not in themselves predictive, however refractory disease is associated with a worse prognosis.[1] Those with a severe ADAMTS-13 deficiency are more likely to have a relapsing course.[1][77][78] 

Use of this content is subject to our disclaimer