Trombotic microangiopathy – a nephrologists‘ view

Česká verzia

Authors: J. Vachek ¹; O. Zakiyanov ¹; V. Motáň ²; V. Tesař ¹
Authors place of work: Klinika nefrologie 1. LF UK a VFN v Praze ¹; Transfuzní oddělení, Oddělení klinické hematologie, Nemocnice České Budějovice a. s. ²
Published in the journal: Gastroent Hepatol 2015; 69(3): 262-266
Category: Vybrané kapitoly z vnitřního lékařství: kazuistika
doi: https://doi.org/10.14735/amgh2015262

Summary

Thrombotic microangiopathy (TMA) is a syndrome characterised by haemolytic anaemia with fragmented red blood cells (schistocytes) in peripheral blood, thrombocytopenia caused by increased platelet aggregation and consumption, resulting in a disturbance of microcirculation caused by a formation of microthrombi. Vascular occlusion causes tissue and organ ischaemia that results in functional disorders of various organs, mainly the central nervous system, kidneys, heart, lungs and gut. Idiopathic TMAs include two etiologically distinct categories known as thrombotic thrombocytopenic purpura and haemolytic uremic syndrome. Secondary TMAs may be caused by certain drugs, infections, malignancies, systemic diseases, or associated with pregnancy. TMA is a rare disease; the reported incidence rates of idiopathic TMA range from one to four patients per million persons year. However, due to the heterogeneity of clinical symptoms, any physician can come across patients suffering from TMA. Timely diagnosis and appropriate treatment are crucial for the outcome of the patients, otherwise the affected patients are threatened with rapid progressive decline of vital functions. This article aims to provide a brief overview of thrombotic microangiopathies and highlights certain novel diagnostic and therapeutic approaches. In addition, the article helps increase general awareness of these diseases, specifically in view of possible difficulties in diagnosis and classification of clinical categories of TMAs.

Key words:
haemolytic uremic syndrome –⁠ thrombotic thrombocytopenic purpura –⁠ thrombocytopenia –⁠ haemolytic anaemia –⁠ plasma exchange –⁠ eculizumab

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE „uniform requirements“ for biomedical papers.

Submitted:
10. 6. 2015

Accepted:
17. 6. 2015

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