Patients with aHUS can be at continuous risk of the life-threatening consequences of unpredictable complement-mediated TMA1,2

Chronic, uncontrolled complement activity in aHUS leads to ongoing endothelial injury, organ damage, and sudden death2,3

aHUS is a disease associated with chronic risk of complement-mediated thrombotic microangiopathy (TMA) and life-threatening consequences1,2

aHUS is defined as a disease that manifests with1

  • The clinical characteristics of TMA (thrombocytopenia, microangiopathic hemolysis, and symptoms of organ dysfunction)
  • ADAMTS13 activity >5% (to rule out severe ADAMTS13 deficiency)
  • Absence of positive STEC test (to rule out STEC-HUS)

Over 70% of adult patients with aHUS are at risk for end-stage renal disease (ESRD) and death4,*
* At last follow-up, 71% (89/125) of adult patients had progressed to ESRD or death.4

Patients without severe ADAMTS13 deficiency do not have a significant clinical benefit from plasma exchange (PE)5

Used with permission from Li A, et al. Transfusion. 2016;56(8):2069-2077. © 2016 AABB.

Complement dysregulation and TMA persist in patients with aHUS on PE/PI (plasma exchange/plasma infusion), even if there is a transient impact on platelet count and LDH levels2,6
  • According to published guidelines of the American Society for Apheresis (ASFA), PE/PI management for aHUS received a Grade 2C (weak recommendation with low/very low-quality evidence) and Category III (optimum role not established)7
  • No randomized, controlled trials have evaluated the role of PE/PI in the management of aHUS7

Patients with aHUS remain at risk of impaired renal function and death, regardless of hematological improvement after PE/PI1

1. Laurence J, et al. Clin Adv Hematol Oncol. 2016;14 Suppl 11(11):2-15. 2. Legendre CM, et al. N Engl J Med. 2013;368(23):2169-2181. 3. Noris M, et al. Nat Rev Nephrol. 2012;8(11):622-633. 4. Fremeaux-Bacchi V, et al. Clin J Am Soc Nephrol. 2013;8(4):554-562. 5. Li A, et al. Transfusion. 2016;56(8):2069-2077. 6. Cofiell R, et al. Blood. 2015;125(21):3253-3262. 7. Schwartz J, et al. J Clin Apher. 2016;31(3):149-338.