Thrombotic thrombocytopenic purpura: the role of ADAMTS13 assay in clinical practice [Trombotak trombocitopenična purpura: uloga testa ADAMTS13 proteaze u kliničkoj praksi]

Duraković, Nadira and Radonić, Radovan and Gašparović, Vladimir (2010) Thrombotic thrombocytopenic purpura: the role of ADAMTS13 assay in clinical practice [Trombotak trombocitopenična purpura: uloga testa ADAMTS13 proteaze u kliničkoj praksi]. Collegium Antropologicum, 34 (3). pp. 1087-1091. ISSN 0350-6134

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Abstract

Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by disseminated thrombotic occlusions of the microcirculation. Identification of ADAMTS13 protease and its place in the pathophysiology of TTP led to better understanding of the disease and better survival for the diseased. Here we show a case report of a patient that had a normal ADAMTS13protease activity and an unusual clinical presentation and utilize that case to highlight how the absence of a severe ADAMTS13 protease deficiency does not preclude a diagnosis of TTP and how early initiation and continuation of plasma exchange therapy can lead to a positive outcome, even in a severely ill patient. Even though ADAMTS13 protease determination has no immediate influence on the decision whether or not to start the plasma exchange therapy, it has great impact on future management of the patient and should be determined whenever possible.

Abstract in Croatian

Trombotska trombocitopenična purpura (TTP) je bolest karakterizirana diseminiranim trombotskim okluzijama mikrocirkulacije. Identifikacija ADAMTS13 proteaze i njenog mjesta u patofiziologiji bolesti dovelo je do boljeg razumijevanja bolesti i boljeg preživljenja oboljelih. Ovaj rad je prikaz slučaja bolesnica s normalnom razinom ADAMTS13 aktivnosti i neobičnom kliničkom prezentacijom uz pomoć kojega želimo naglasiti kako odsustvo teškog pomanjkanja ADAMTS13 aktivnosti ne izuzima dijagnozu TTP i kako rano započinjanje i nastavak terapije izmjenom plazme može dovesti do pozitivnog ishoda, čak i u teško oboljelih. Iako određivanje nedostatka ADAMTS13 aktivnosti nema trenutni utjecaj na odluku da li ili ne započeti terapiju izmjenom plazme, ima veliki utjecaj na daljnje terapijske odluke i trebala bi biti pokušana kad god je to moguće.

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