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2013, Number 1

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Rev Fac Med UNAM 2013; 56 (1)

Thrombotic thrombocytopenic purpura. A case and review of its physiopathology

Salazar MMF, Reyes CMM

Language: Spanish
References: 22
Page: 30-38
PDF size: 385.31 Kb.


ABSTRACT

We report a case compatible with thrombotic thrombocytopenic purpura, autopsy findings and make a brief review of the literature. 19 year old woman with HELLP syndrome in her previous pregnancy who presented with neurological signs, thrombocytopenia and microangiopathic haemolytic anemia until her pass away fifteen days after being admitted to the hospital. Autopsy findings showed multiple thrombi in small sized vessels of several organs. Thrombotic thrombocitopenic purpura is a rare disease with a morphological expression featured of many microthrombi in the terminal arterioles of several vital structures. Moschcowitz was the first to inform multiple hyaline thrombi as the primordial finding of a partial autopsy case. He proposed that “a powerful poison with both agglutinative and hemolytic properties” was the causative agent but it was identified years later as unusually large fragments of Von Willebrand factor caused by a deficiency of ADAMTS13, a newly discovered metalloproteinase.


REFERENCES

  1. Moschcowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc N Y Pathol Soc. 1924;24:21-4.

  2. Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. Arch Intern Med. 1925;36:89-93.

  3. De la Rubia J, Contreras E, Del Río-Garma J. Púrpura trombocitopénica trombótica. Med Clin (Barc). 2011. doi:10.1016/j.medcli.2010.02.011

  4. Sadler JE. Von Willebrand, ADAMTS13, and thrombotic thrombocytopenic purpura. Blood. 2008; 112(1):11-8.

  5. Moake JL. Von Willebrand, ADAMTS-13, and thrombotic thrombocytopenic purpura. Seminars in Hematology. 2001;41(1):4-14.

  6. Singer K, Bornstein FP, Wile SA. Thrombotic thrombocytopenic purpura. Hemorrhagic diathesis with generalized platelet thromboses. Blood New York. 1947;2:542-4.

  7. Ma A. Molecular Basis of Disorders of Hemostasis and Thrombosis. In: Molecular Pathology Library, 2009, 1, Volume 4, Molecular Pathology of Hematolymphoid Diseases, Part 3, pp511-28.

  8. Fujimura Y, Matsumoto M, Yagi H. Thrombotic Microangiopathy. Rinsho Shinkeigaku. 2006;46(11):902-5.

  9. Zheng XL, Sadler JE. Pathogenesis of thrombotic microangiopathies. Annu Rev Pathol. 2008;3:249-77.

  10. Allford SL, Machin SJ. Current understanding of the pathophysiology of thrombotic thrombocytopenic purpura. J Clin Pathol. 2000;53:497-501.

  11. Manea M, Karpman D. Molecular basis of ADAMTS13 dysfunction in thrombotic thrombocytopenic purpura. Pediatr Nephrol. 2009;24:447-58.

  12. Han-Mou Tsai. Deficiency of ADAMTS13 in thrombotic thrombocytopenic purpura. International Journal of Hematology. 2002;76 Suppl 2:132-18.

  13. Banno F, Miyata T. Biology of an Antithrombotic Factor-ADAMTS13. In: Tanaka K, Davie EW. Recent Advances in Thrombosis and Hemostasis. Springer; 2008. Pp 162-176.

  14. Halevy D, Radhakrishnan J, Markowitz G, et al. Thrombotic microangiopathies. Crit Care Clin. 2002);18:309-20.

  15. Ruggenenti P, Chiurchiu C, Remuzzi G. Microangiopatía trombótica, síndrome urémico hemolítico y púrpura trombocitopénica trombótica. Disponible en: http://www.uninet.edu/cin2001-old/conf/remuzzi/remuzzi.html

  16. Taleb A, Ayatollah S, Hamid M, et al. Thrombotic Thrombocytopenic Purpura associated with Clopidogrel: a case report and review of the literature. J Res Med Sci. 2011;16(3): 353-7.

  17. Fabris F, Luzzattto G. A paradoxical side-effect of antiaggregating treatment with ticlopidine: the Moschowitz syndrome. Haematologica. 1999;84(8):760-1.

  18. Pinto JA, Suárez De Claudia B, Rumenoff Lila. Lesiones miocárdicas en púrpura trombocitopénica trombótica (PTT). Estudio clinicopatológico de 11 casos de autopsia. Acta Anatomopatológica. 1999;3(1):3-11.

  19. Delgado C, Flores C, Silva C. Aspectos neurológicos del púrpura trombótico trombocitopénico. Presentación de un caso. Rev Med Chile. 2006;134:348-52.

  20. Craig JM, Gitlin M. The Nature of the Hyaline Thrombi in Thrombotic Thrombocytopenic Purpura. Am J Pathol. 1957;33(2):251-65.

  21. Ganong WF. Biophysical considerations of blood and lymph flow. In: Ganong WF. “Review of Medical Physiology. 21st Edition McGraw-Hill 2003. Section VI Circulation Chapter 30 Dunamics of Blood & Lympph Flow; pp. 584-9.

  22. Hewwit P. Principio de Bernoulli. En: Hewitt P. Conceptos de Física. 1° Edición Sexta Reimpresión Limusa 1997. Parte III Propiedades de la Materia. Capítulo 12 Gases y plasmas; pp. 231-236.




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