Retrospective analysis of patients with thrombotic thrombocytopenic purpura treated at the University Hospital Center Zagreb

master's thesis

• Title (croatian): Retrospektivna analiza bolesnika s trombotičnom trombocitopeničnom purpurom liječenim u KBC Zagreb
• Author: Kiarash Pour Modjib
• Mentor: Dražen Pulanić (mentor)
• Committee member: Dražen Pulanić (predsjednik povjerenstva)
• Committee member: Nadira Duraković (član povjerenstva)
• Committee member: Martina Lovrić Benčić (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; Zagreb
• Defense date and country: 2023-07-13, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Internal Medicine

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic hemolytic anemia with thrombocytopenia caused by low ADAMTS13. There is the acquired and the hereditary type. In the acquired type there is immune-mediated ADAMTS13 deficiency, caused by autoantibodies against ADAMTS13, and the hereditary TTP is caused by compound heterozygote or homozygote mutations in ADAMTS13 gene. The clinical picture of acute TTP presents with microangiopathic hemolytic anemia, thrombocytopenia, usually mild kidney impairment, neurological symptoms and sometimes fever. Since TTP is an emergency, which can end up in stroke, multiorgan failure and death, quick diagnosis and intervention is needed.

In this work retrospective analysis of 10 patients with TTP who were followed up in the Hematology outpatient clinic of the University Hospital Center Zagreb from 2016-2022 was performed. Two patients had hereditary and 8 patients had acquired TTP.

This work confirmed already existing facts about the disease and presents results about age and gender distribution, clinical symptoms, laboratory parameters, etiological factors, treatment outcome and long – term sequelae in these patients.

Abstract (croatian)

Trombotična trombocitopenijčna purpura (TTP) je mikroangiopatska hemolitička anemija s trombocitopenijom uzrokovana niskim ADAMTS13. Postoji stečeni i nasljedni tip. U stečenom tipu postoji imunološki posredovani nedostatak ADAMTS13, uzrokovan autoantitijelima protiv ADAMTS13, a nasljedni TTP uzrokovan je složenim heterozigotnim ili homozigotnim mutacijama u genu ADAMTS13. Klinička slika akutne TTP manifestira se mikroangiopatskom hemolitičkom anemijom, trombocitopenijom, obično blagim oštećenjem bubrega, neurološkim simptomima i ponekad vrućicom. Budući da je TTP hitan slučaj, koji može završiti moždanim udarom, multiorganskim zatajenjem i smrću, potrebna je brza dijagnoza i intervencija.

U ovom radu provedena je retrospektivna analiza 10 bolesnika s TTP-om koji su praćeni u hematološkoj ambulanti KBC-a Zagreb od 2016.-2022. Dva bolesnika imala su nasljednu, a 8 bolesnika stečenu TTP.

Ovim radom potvrđene su već postojeće činjenice o bolesti te su prikazani rezultati o dobnoj i spolnoj distribuciji, kliničkim simptomima, laboratorijskim parametrima, etiološkim čimbenicima, ishodu liječenja i dugoročnim posljedicama u ovih bolesnika.

• Keywords: thrombotic thrombocytopenic purpura; microangiopathic hemolytic anemia; ADAMTS13; thrombocytopenia
• Keywords (croatian): trombotična trombocitopenična purpura; mikroangiopatska hemolitička anemija; ADAMTS13; trombocitopenija
• Language: english
• URN:NBN: urn:nbn:hr:105:777383
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2023-09-01 11:57:50