A guide to differential diagnosis and management of TMA

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Overlapping disorders highlight the importance of a management algorithm for TMAs 2,3

Persistent TMA, despite conventional treatment warrants further investigation:

  • Treat. On TMA presentation, treat the initial disease process adequately 2,3
  • Assess. If treatment fails to resolve the TMA, follow up with differential diagnosis of TMA 2,3
  • Test. To determine if the underlying cause of the TMA is aHUS, STEC-HUS or TTP 2,3
TREAT > ASSESS > TEST
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Adapted from Asif et al, 2016.

69% of aHUS patients experienced their first manifestation of aHUS while experiencing one of the following complement-amplifying conditions (n=191)4

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Study design: A Registry analysis of 273 adult and paediatric patients with aHUS. The focus of the analysis was on complement abnormalities and their role in predicting clinical phenotype and response to treatment. The analysis included the incidence of comorbidity at disease onset.

Adapted from Noris et al, 2010.

aHUS is a rare, life-threatening, systemic disease 1-3

When unrecognised or inappropriately treated, it has a high degree of morbidity and mortality. 3 Early diagnosis and treatment is essential to avoid end-stage renal disease or death. 3,5

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References:
  1. Laurence J. Clinical Advances in Hematology & Oncology. 2012;10(10)1-11.
  2. Asif A, et al. J Nephrol. 2016 DOI: 10.1007/s40620-016-0357-7
  3. Laurence J, et al. Clinical Advances in Hematology & Oncology. 2016;14(11)1-15.
  4. Noris M, et al. Clin J Am Soc Nephrol. 2010;5:1844-1859.
  5. Licht C, et al. Kidney International. 2015;87:1061–1073.