Correct!
5. All of the above

Wu et al (8) reported that ADAMTS13 response to early plasma exchange therapy in patients with acquired TTP had prognostic value. In their study of 19 patients, recovery of ADAMTS13 activity to more than 10% within 7 days was significantly associated with a timely clinical response. In contrast, patients whose ADAMTS13 level failed to exceed 10% by 7 days tended to experience TTP exacerbation, treatment refractoriness, or death.

Our patient had a dramatic clinical response to plasma exchange and Rituximab (5). She was deemed stable for discharge with Rituximab, outpatient plasmapheresis and follow up.

References

  1. Brodsky RA. Diagnosis of hemolytic anemia in adults. UpToDate. October 12, 2021. Available at: https://www.uptodate.com/contents/diagnosis-of-hemolytic-anemia-in-adults (accessed 12/3/21, requires subscription).
  2. Hubbell FA, Greenfield S, Tyler JL, Chetty K, Wyle FA. The impact of routine admission chest x-ray films on patient care. N Engl J Med. 1985 Jan 24;312(4):209-13. [CrossRef] [PubMed]
  3. Wun T. Thrombotic Thrombocytopenic Purpura (TTP). Medscape. May 25, 2021. Available at: https://emedicine.medscape.com/article/206598-overview (accessed 12/3/21).
  4. ZE, Okunji C, Hunt RC, Gupta T, Allen CE, Plum E, Blaisdell A, Grigoryan V, Geetha S, Fathke R, Soejima K, Kimchi-Sarfaty C. Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease. PLoS One. 2009 Aug 5;4(8):e6506. [CrossRef] [PubMed]
  5. Masias C, Vasu S, Cataland SR. None of the above: thrombotic microangiopathy beyond TTP and HUS. Blood. 2017 May 25;129(21):2857-2863. [CrossRef] [PubMed]
  6. Shah N, Rutherford C, Matevosyan K, Shen YM, Sarode R. Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP). Br J Haematol. 2013 Nov;163(4):514-9. [CrossRef] [PubMed]
  7. Michael M, Elliott EJ, Craig JC, Ridley G, Hodson EM. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis. 2009 Feb;53(2):259-72. [CrossRef] [PubMed]
  8. Wu N, Liu J, Yang S, Kellett ET, Cataland SR, Li H, Wu HM. Diagnostic and prognostic values of ADAMTS13 activity measured during daily plasma exchange therapy in patients with acquired thrombotic thrombocytopenic purpura. Transfusion. 2015 Jan;55(1):18-24. [CrossRef] [PubMed]
  9. Gutterman LA, Kloster B, Tsai HM. Rituximab therapy for refractory thrombotic thrombocytopenic purpura. Blood Cells Mol Dis. 2002 May-Jun;28(3):385-91. [CrossRef] [PubMed]

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