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

Published on Feb 1, 2018in Transfusion and Apheresis Science 1.41
· DOI :10.1016/j.transci.2018.02.012
Emre Tekgündüz8
Estimated H-index: 8
,
Mehmet Yilmaz24
Estimated H-index: 24
(University of Gaziantep)
+ 25 AuthorsFatih Demirkan11
Estimated H-index: 11
(Dokuz Eylül University)
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Abstract
Abstract Thrombotic 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.
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References28
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Published on Oct 1, 2017in Ndt Plus
Vicky Brocklebank6
Estimated H-index: 6
(Newcastle upon Tyne Hospitals NHS Foundation Trust),
David J. Kavanagh65
Estimated H-index: 65
(Newcastle upon Tyne Hospitals NHS Foundation Trust)
14 Citations Source Cite
Published on Aug 1, 2017in Chest 9.66
Elie Azoulay83
Estimated H-index: 83
,
Paul Knoebl15
Estimated H-index: 15
(Medical University of Vienna)
+ 8 AuthorsJulia Wendon63
Estimated H-index: 63
(University of Cambridge)
A typical hemolytic uremic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP) and other causes or conditions with thrombotic microangiopathy (TMA), such as disseminated intravascular coagulation or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarize available da...
4 Citations Source Cite
Published on Aug 1, 2017in Therapeutic Apheresis and Dialysis 1.08
Alexander Åkesson1
Estimated H-index: 1
(Lund University),
Eva Zetterberg2
Estimated H-index: 2
(Lund University),
Jenny Klintman5
Estimated H-index: 5
(Lund University)
Complement-mediated atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with high mortality and morbidity. Renal biopsies often indicate thrombotic microangiopathy (TMA). The condition is caused by an excessive activation of the alternative pathway leading to depositions of membrane attack complexes (MAC) on host cells. It may depend on mutations in complement components and regulatory proteins, or the formation of complement-specific antibodies. Mainly, an environmental trigg...
2 Citations Source Cite
Published on Jul 20, 2017in Blood 16.56
Samuel Merrill2
Estimated H-index: 2
,
Zachary D. Brittingham1
Estimated H-index: 1
+ 3 AuthorsRobert A. Brodsky44
Estimated H-index: 44
23 Citations Source Cite
Published on Jul 1, 2017in Seminars in Thrombosis and Hemostasis 3.40
Pavan K. Bendapudi10
Estimated H-index: 10
(Harvard University),
Vivek A. Upadhyay3
Estimated H-index: 3
(Harvard University)
+ 2 AuthorsRobert S. Makar15
Estimated H-index: 15
(Harvard University)
Thrombotic microangiopathies (TMAs) are a group of rare but potentially lethal hematologic disorders characterized by platelet-rich thrombi in the microvasculature. In evaluating patients with TMA, it is crucial to identify those who have thrombotic thrombocytopenic purpura (TTP), a subtype of TMA that occurs due to a severe deficiency in ADAMTS13 ( a d isintegrin a nd m etalloprotease with t hrombo s pondin type 1 motif, member 13 ). Individuals with TTP require urgent therapeutic plasma exchan...
5 Citations Source Cite
Published on Mar 1, 2017in Nephrology Dialysis Transplantation 4.20
Teresa Cavero5
Estimated H-index: 5
,
Cristina Rabasco5
Estimated H-index: 5
+ 25 AuthorsMiquel Blasco10
Estimated H-index: 10
Work in this report was funded by the Instituto de Salud Carlos III: REDinREN (RD 016/009 Feder Funds), the Fondo de Investigaciones Sanitarias (13/02502 and ICI14/00350), the Ministerio de Economia y Competitividad (SAF2015-66287R) and the Autonomous Region of Madrid (S2010/BMD-2316; Grupo de Investigacion Complemento-CM). SRdeC is funded by the Seventh Framework Programme European Union Project EURenOmics (305608)
23 Citations Source Cite
Published on Feb 1, 2017in Journal of Thrombosis and Haemostasis 4.66
Marie Scully27
Estimated H-index: 27
(UCLH: University College Hospital),
Spero R. Cataland23
Estimated H-index: 23
(OSU: Ohio State University)
+ 9 AuthorsE. Sadler1
Estimated H-index: 1
(WashU: Washington University in St. Louis)
Essentials An international collaboration provides a consensus for clinical definitions. This concerns thrombotic microangiopathies and thrombotic thrombocytopenic purpura (TTP). The consensus defines diagnosis, disease monitoring and response to treatment. Requirements for ADAMTS-13 are given. SummaryBackground Thrombotic thrombocytopenic purpura (TTP) and hemolytic–uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic microangiopathy (TMA) is a broad pathophysiologic...
63 Citations Source Cite
Published on Jan 12, 2017in Ndt Plus
Manuel Macia1
Estimated H-index: 1
,
Fernando de Alvaro Moreno1
Estimated H-index: 1
+ 4 AuthorsNils Heyne17
Estimated H-index: 17
(University of Tübingen)
19 Citations Source Cite
Published on Dec 1, 2016in Hematology 1.32
Vahid Afshar-Kharghan1
Estimated H-index: 1
5 Citations Source Cite
Laurence J1
Estimated H-index: 1
,
Haller H1
Estimated H-index: 1
+ 3 AuthorsRodriguez de Cordoba S1
Estimated H-index: 1
11 Citations
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