A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study

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dc.contributor.authorTekgündüz E.
dc.contributor.authorYılmaz M.
dc.contributor.authorErkurt M.A.
dc.contributor.authorKiki I.
dc.contributor.authorKaya A.H.
dc.contributor.authorKaynar L.
dc.contributor.authorAlacacioglu I.
dc.contributor.authorCetin G.
dc.contributor.authorOzarslan I.
dc.contributor.authorKuku I.
dc.contributor.authorSincan G.
dc.contributor.authorSalim O.
dc.contributor.authorNamdaroglu S.
dc.contributor.authorKarakus A.
dc.contributor.authorKarakus V.
dc.contributor.authorAltuntas F.
dc.contributor.authorSari I.
dc.contributor.authorOzet G.
dc.contributor.authorAydogdu I.
dc.contributor.authorOkan V.
dc.contributor.authorKaya E.
dc.contributor.authorYildirim R.
dc.contributor.authorYildizhan E.
dc.contributor.authorOzgur G.
dc.contributor.authorOzcebe O.I.
dc.contributor.authorPayzin B.
dc.contributor.authorAkpinar S.
dc.contributor.authorDemirkan F.
dc.date.accessioned2024-07-22T08:09:57Z
dc.date.available2024-07-22T08:09:57Z
dc.date.issued2018
dc.description.abstractThrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1­75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE. © 2018 Elsevier Ltd
dc.identifier.DOI-ID10.1016/j.transci.2018.02.012
dc.identifier.issn14730502
dc.identifier.urihttp://akademikarsiv.cbu.edu.tr:4000/handle/123456789/15022
dc.language.isoEnglish
dc.publisherElsevier Ltd
dc.subjectADAMTS13 Protein
dc.subjectAdolescent
dc.subjectAdult
dc.subjectAged
dc.subjectAged, 80 and over
dc.subjectAutoantibodies
dc.subjectFemale
dc.subjectFollow-Up Studies
dc.subjectHemolytic-Uremic Syndrome
dc.subjectHumans
dc.subjectMale
dc.subjectMiddle Aged
dc.subjectPlasma Exchange
dc.subjectRetrospective Studies
dc.subjectTurkey
dc.subjecteculizumab
dc.subjectimmunosuppressive agent
dc.subjectrituximab
dc.subjectsteroid
dc.subjectvon Willebrand factor cleaving proteinase
dc.subjectADAMTS13 protein, human
dc.subjectautoantibody
dc.subjectvon Willebrand factor cleaving proteinase
dc.subjectadolescent
dc.subjectadult
dc.subjectaged
dc.subjectArticle
dc.subjectcohort analysis
dc.subjectcontrolled study
dc.subjectdisease exacerbation
dc.subjectenzyme activity
dc.subjectenzyme blood level
dc.subjectfemale
dc.subjectfollow up
dc.subjecthemolytic uremic syndrome
dc.subjecthospital admission
dc.subjecthuman
dc.subjectimmunosuppressive treatment
dc.subjectkidney failure
dc.subjectmajor clinical study
dc.subjectmale
dc.subjectpathophysiology
dc.subjectplasma exchange
dc.subjectplasma volume
dc.subjectrelapse
dc.subjectremission
dc.subjectretrospective study
dc.subjectsteroid therapy
dc.subjectthrombotic thrombocytopenic purpura
dc.subjecttreatment response
dc.subjectTurkey (republic)
dc.subjectblood
dc.subjectclinical trial
dc.subjecthemolytic uremic syndrome
dc.subjectimmunology
dc.subjectmiddle aged
dc.subjectmortality
dc.subjectmulticenter study
dc.subjectpathology
dc.subjectplasma exchange
dc.subjectturkey (bird)
dc.subjectvery elderly
dc.titleA multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study
dc.typeArticle

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