THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) AND MODERN APPROACH TO ITS INVESTIGATION AND TREATMENT

  • K. Ukleba Department of Clinical Medicine, Medical faculty, Akaki Tsereteli state University Kutaisi, Georgia
  • L. Gvetadze Department of Clinical Medicine, Medical faculty, Akaki Tsereteli state University Kutaisi, Georgia
Keywords: Thrombocytopenic Purpura, Microangiopatic hemolytic anemia, ADAMTS13

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving protease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies, but rarely, it is inherited via mutations of the ADAMTS13 gane. The first acute episode of TTP usually occurs during adulthood, with a predominant anti – ADAMTS13 autoimmune etiology. In rare cases, however, TTP begins as soon as childhood, with frequent inherited forms. TTP is 2 – fold more frequent in women, and its outcome is characterized by a relapsing tendency.

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Published
2019-03-31
Citations
How to Cite
K. Ukleba, & L. Gvetadze. (2019). THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) AND MODERN APPROACH TO ITS INVESTIGATION AND TREATMENT. Science Review, (3(20), 12-13. https://doi.org/10.31435/rsglobal_sr/31032019/6381
Section
Medicine