Терапевтический архив (Jul 2019)

Clinical observation of a patient with thrombotic thrombocytopenic purpura with renal and intestinal lesions

  • A G Stolyar,
  • P V Avdonin,
  • I E Valamina,
  • E S Stolyarevich

DOI
https://doi.org/10.26442/00403660.2019.07.000131
Journal volume & issue
Vol. 91, no. 7
pp. 106 – 110

Abstract

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The article presents a brief description of a rare disease - thrombotic thrombocytopenic purpura (Moshkovits - disease), which is based on the deficiency of ADAMTS-13 metalloproteinase, leading to the development of thrombotic microangiopathy with the defeat of vital organs. The article also describes the clinical observation of a patient with the Moshkovits - disease. The features of the above observation are involvement in the pathological process of the kidneys and intestines, while in the classical descriptions of the disease there is a predominant lesion of the Central nervous system, as well as the genetic form of the disease.

Keywords