Acquired thrombotic thrombocytopenic purpura

Czech version

Authors: M. Lánská; P. Žák
Authors‘ workplace: IV. interní hematologická klinika LF UK a FN Hradec Králové
Published in: Transfuze Hematol. dnes,30, 2024, No. S1, p. 48-54.
Category:
doi: https://doi.org/10.48095/cctahd2024suppl1S48

Overview

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease belonging to the group of thrombotic microangiopathies (TMA). It is characterised by the presence of microangiopathic haemolytic anaemia, severe thrombocytopenia, and ischaemic end-organ damage. The underlying cause of the disease is a severe deficiency of von Willebrand factor (vWF) cleaving metalloprotease. The enzyme deficiency causes the accumulation of large multimers of vWF to which platelets bind actively, with subsequent formation of microthrombi in the microcirculation. The most important test for the diagnosis of TTP is reduced ADAMTS13 enzyme activity below 10% (0.1 IU/ml). Treatment of TTP should be initiated as early as possible when it is suspected, often before the ADAMTS13 activity results are known. The current standard of care for acute attacks of acquired TTP includes therapeutic plasma exchange, immunosuppression, and caplacizumab. A rapid diagnosis of TTP, including ADAMTS13 activity testing, and early initiation of comprehensive treatment are critical to treatment success. Long-term monitoring of disease activity, including monitoring of ADAMTS13 activity, is also a necessary part of the treatment

Keywords:

Steroids – thrombotic thrombocytopenic purpura – therapeutic plasma exchange – rituximab – ADAMTS13 – caplacizumab

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PODÍL AUTORŮ NA RUKOPISU

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PZ – korekce a revize rukopisu

PROHLÁŠENÍ AUTORŮ

Autoři práce prohlašují, že v souvislosti s tématem, vznikem a publikací tohoto článku nejsou ve střetu zájmů a vznik ani publikace článku nebyly podpořeny žádnou farmaceutickou firmou.

Do redakce doručeno dne: 18. 1. 2024.

Přijato po recenzi dne: 3. 6. 2024.

MU Dr. Miriam Lánská, Ph.D.

IV. interní hematologická klinika

FN Hradec Králové

Sokolská 581

e-mail: miriam.lanska@fnhk.cz