Thrombotic thrombocytopenic purpura pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

The exact pathogenesis of thrombotic thrombocytopenic purpura (TTP) is not fully understood. It is thought that TTP is caused by the deficiency of a plasma metalloprotease, ADAMTS13.

Pathophysiology

Genetics

Genes involved in the pathogenesis of TTP include:[4]

Microscopic Pathology

On microscopic histopathological analysis findings of TTP include:

  • Granular (muddy brown) casts
  • Characteristic fibrin thrombi in glomerular and interstitial capillaries
  • Slough into tubular lumen
High magnification microscopy of HUS Source:By Nephron [CC BY-SA 3.0 (https://creativecommons.org/licenses/by-sa/3.0) or GFDL (http://www.gnu.org/copyleft/fdl.html)], from Wikimedia Commons

References

  1. 1.0 1.1 Tsai HM (January 2010). "Pathophysiology of thrombotic thrombocytopenic purpura". Int. J. Hematol. 91 (1): 1–19. doi:10.1007/s12185-009-0476-1. PMC 3159000. PMID 20058209.
  2. Porter S, Clark IM, Kevorkian L, Edwards DR (February 2005). "The ADAMTS metalloproteinases". Biochem. J. 386 (Pt 1): 15–27. doi:10.1042/BJ20040424. PMC 1134762. PMID 15554875.
  3. Zheng XL (June 2013). "Structure-function and regulation of ADAMTS-13 protease". J. Thromb. Haemost. 11 Suppl 1: 11–23. doi:10.1111/jth.12221. PMC 3713533. PMID 23809107.
  4. Conboy E, Partain PI, Warad D, Kluge ML, Arndt C, Chen D, Rodriguez V (January 2018). "A Severe Case of Congenital Thrombotic Thrombocytopenia Purpura Resulting From Compound Heterozygosity Involving a Novel ADAMTS13 Pathogenic Variant". J. Pediatr. Hematol. Oncol. 40 (1): 60–62. doi:10.1097/MPH.0000000000000895. PMID 28678087.
  5. Fujimura Y, Matsumoto M, Isonishi A, Yagi H, Kokame K, Soejima K, Murata M, Miyata T (July 2011). "Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan". J. Thromb. Haemost. 9 Suppl 1: 283–301. doi:10.1111/j.1538-7836.2011.04341.x. PMID 21781265.

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