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 1.75
· 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)
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
Published on Jun 1, 2015in Hematology-oncology Clinics of North America 3.10
C. John Sperati11
Estimated H-index: 11
(Johns Hopkins University School of Medicine),
Alison R. Moliterno23
Estimated H-index: 23
(Johns Hopkins University School of Medicine)
16 Citations Source Cite
Published on Jan 1, 2016in Internal Medicine Journal 1.78
Piers Blombery10
Estimated H-index: 10
(Monash University),
Leah Kivivali1
Estimated H-index: 1
(Monash University)
+ 6 AuthorsSolomon Cohney17
Estimated H-index: 17
(Monash University)
Background Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA). In 2009, the Australian TTP/TMA registry was established to collect data on patients presenting with TTP/TMA throughout Australia. Aim To summarise information on the diagnosis and management of patients with TTP collected in the first 5 years (2009–2014) of the Australian TTP registry. Methods Registry data from June 2009 to October 2014 were reviewed. Results Fifty-seven patients ...
10 Citations Source Cite
Published on Dec 1, 2015in British Journal of Haematology 5.13
Pavan K. Bendapudi10
Estimated H-index: 10
(Harvard University),
Ang Li4
Estimated H-index: 4
(Harvard University)
+ 5 AuthorsRobert S. Makar15
Estimated H-index: 15
(Harvard University)
Summary The Harvard TMA Research Collaborative is a multi-institutional registry-based effort to study thrombotic microangiopathies (TMA). Laboratory and clinical parameters were recorded for 254 cases of suspected autoimmune thrombotic thrombocytopenic purpura (TTP). Patients with severe ADAMTS13 deficiency (activity ≤10%, N = 68) were more likely to be young, female and without a history of cancer treatment or transplantation. While all patients with severe deficiency were diagnosed with autoi...
19 Citations Source Cite
Published on Jun 12, 2014in Blood 15.13
Spero R. Cataland23
Estimated H-index: 23
(Ohio State University),
V. Michael Holers66
Estimated H-index: 66
(University of Colorado Boulder)
+ 2 AuthorsHaifeng M. Wu17
Estimated H-index: 17
(Ohio State University)
Atypical hemolytic uremic syndrome (aHUS) is characterized by dysregulated complement activity, the development of a thrombotic microangiopathy (TMA), and widespread end organ injury. aHUS remains a clinical diagnosis without an objective laboratory test to confirm the diagnosis. We performed a
82 Citations Source Cite
Published on Sep 5, 2014in Turkish Journal of Hematology 0.65
Mustafa N. Yenerel1
Estimated H-index: 1
Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA). It has an unfavorable outcome with death rates as high as 25% during the acute phase and up to 50% of cases progressing to end-stage renal failure. Uncontrolled complement activation through the alternative pathway is thought to be the main underlying pathopysiology of aHUS and corresponds to all the deleterious findings of the disease. Thrombotic thrombocytopenic purpura (TTP) and Shiga toxin-associate...
4 Citations Source Cite
Published on Apr 23, 2010in PLOS ONE 2.77
Paul Coppo34
Estimated H-index: 34
,
Michaël Schwarzinger19
Estimated H-index: 19
(French Institute of Health and Medical Research)
+ 22 AuthorsElie Azoulay82
Estimated H-index: 82
(University of Paris)
Severe ADAMTS13 deficiency occurs in 13% to 75% of thrombotic microangiopathies (TMA). In this context, the early identification of a severe, antibody-mediated, ADAMTS13 deficiency may allow to start targeted therapies such as B-lymphocytes-depleting monoclonal antibodies. To date, assays exploring ADAMTS13 activity require skill and are limited to only some specialized reference laboratories, given the very low incidence of the disease. To identify clinical features which may allow to predict r...
139 Citations Source Cite
Published on Apr 17, 2014in Blood 15.13
Spero R. Cataland23
Estimated H-index: 23
,
Haifeng M. Wu17
Estimated H-index: 17
(Ohio State University)
Published data demonstrating the efficacy of complement inhibition therapy in patients with atypical hemolytic uremic syndrome (aHUS) are remarkable in contrast to the historically poor long-term prognosis for aHUS patients treated with plasma-based therapy. Although both aHUS and acquired thrombotic thrombocytopenic purpura (TTP) remain clinical diagnoses, an increased understanding of both conditions has improved our ability to differentiate aHUS from acquired TTP. These same data have also de...
64 Citations Source Cite
Published on Jun 4, 2015in Blood 15.13
Eleni Gavriilaki11
Estimated H-index: 11
,
Xuan Yuan6
Estimated H-index: 6
+ 7 AuthorsRobert A. Brodsky45
Estimated H-index: 45
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by excessive activation of the alternative pathway of complement (APC). Atypical HUS is frequently a diagnosis of exclusion. Differentiating aHUS from other TMAs, especially thrombotic thrombocytopenic
40 Citations Source Cite
Marina Noris58
Estimated H-index: 58
,
Jessica Caprioli17
Estimated H-index: 17
+ 15 AuthorsAnnalisa Sorosina2
Estimated H-index: 2
Background and objectives: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Most childhood cases are caused by Shiga toxin–producing bacteria. The other form, atypical HUS (aHUS), accounts for 10% of cases and has a poor prognosis. Genetic complement abnormalities have been found in aHUS. Design, setting, participants, and measurements: We screened 273 consecutive patients with aHUS for complement abnormalities and stu...
449 Citations Source Cite
Published on Nov 18, 2010in Blood 15.13
James N. George67
Estimated H-index: 67
Thrombotic thrombocytopenic purpura (TTP) is the common name for adults with microangiopathic hemolytic anemia, thrombocytopenia, with or without neurologic or renal abnormalities, and without another etiology; children without renal failure are also described as TTP. The diagnosis of TTP is an indication for plasma exchange treatment, but beginning treatment requires sufficient confidence in the diagnosis to justify the risk of plasma exchange complications. Documentation of a severe deficiency...
295 Citations Source Cite
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