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      Acquired thrombotic thrombocytopenic purpura with possible association with AstraZeneca‐Oxford COVID‐19 vaccine

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          Abstract

          Acquired thrombotic thrombocytopenic purpura is characterized by the microvascular aggregation of platelets and microangiopathic hemolytic anemia causing ischemia of multiple organs including the brain mainly and less likely the kidney and the heart. The disease is caused by severe reduction in the activity of ADAMTS 13 due to presence of inhibitory antibodies.

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          Most cited references18

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          Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.

          Among the syndromes characterised by thrombotic microangiopathy, thrombotic thrombocytopenic purpura is distinguished by a severe deficiency in the ADAMTS13 enzyme. Patients with this disorder need urgent treatment with plasma exchange. Because ADAMTS13 activity testing typically requires prolonged turnaround times and might be unavailable in resource-poor settings, a method to rapidly assess the likelihood of severe ADAMTS13 deficiency is needed.
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            Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients.

            Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction. In early reports the mortality approached 100 percent. A treatment protocol was introduced in 1979 for patients admitted to Johns Hopkins Hospital with the diagnosis of TTP-HUS. Treatment regimens included 200 mg of prednisone a day, for patients with minimal symptoms and no central nervous system symptoms, and prednisone plus plasma exchange, for patients with rapid clinical deterioration who did not improve after 48 hours of prednisone alone and for patients presenting with central nervous system symptoms and rapidly declining hematocrit values and platelet counts. A total of 108 patients were treated, and 91 percent survived. Prednisone alone was judged to be effective in 30 patients with mild TTP-HUS (two relapses and two deaths). Plasma exchange plus prednisone was given to 78 patients with complicated TTP-HUS, resulting in 67 relapses and 8 deaths. Relapses occurred in 22 of 36 patients given maintenance plasma infusions. Neither splenectomy nor treatment with aspirin and dipyridamole was effective in those with a poor response to plasma exchange. None of the 71 patients tested had positive cultures for O157:H7 Escherichia coli. Nine percent of the patients were pregnant, and none gave birth to infants with TTP-HUS. Effective treatment with 91 percent survival is available for patients with TTP-HUS.
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              Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group.

              Thrombotic thrombocytopenic purpura is an uncommon disease with a high mortality rate even with current treatment. The cause of the syndrome and its optimal treatment are unknown. Although both plasma exchange and plasma infusion have been useful treatments, it is not clear which is superior. In this report we describe a prospective randomized trial comparing plasma exchange with plasma infusion for the treatment of thrombotic thrombocytopenic purpura. One hundred two patients with thrombotic thrombocytopenic purpura were randomly assigned to receive either plasma exchange or plasma infusion with fresh-frozen plasma on seven of the first nine days after entry into the trial. The total volume of plasma received by patients undergoing plasma exchange was three times that received by patients undergoing plasma infusion. All the patients also received aspirin and dipyridamole. The outcomes in the two groups were compared at the end of the first treatment cycle (day 9) and after six months. At the end of the first treatment cycle patients receiving plasma exchange had a higher rate of response as defined by an increase in the platelet count (24 of 51 patients) than those who received plasma infusion (13 of 51, P = 0.025). Of the 51 patients treated with plasma exchange, 2 died, whereas 8 of the 51 patients who received plasma infusion died (P = 0.035). After six months the outcome in the plasma-exchange group was still superior, with a response observed in 40 of 51 patients, whereas 25 of 51 patients in the plasma-infusion group responded (P = 0.002). Eleven patients in the plasma-exchange group died, as did 19 patients in the plasma-infusion group (P = 0.036). The overall mortality was 29 percent. Plasma exchange is more effective than plasma infusion in the treatment of thrombotic thrombocytopenic purpura.
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                Author and article information

                Contributors
                mona.alahmad@ku.edu.kw
                Journal
                EJHaem
                EJHaem
                10.1002/(ISSN)2688-6146
                JHA2
                Ejhaem
                John Wiley and Sons Inc. (Hoboken )
                2688-6146
                18 May 2021
                : 10.1002/jha2.219
                Affiliations
                [ 1 ] Microbiology Department Faculty of Medicine Kuwait University Kuwait Kuwait
                [ 2 ] Al Rashed Allergy Centre Ministry of Health Kuwait
                [ 3 ] Hematology Unit Department of Medicine Al Adnan Hospital Ministry of Health Kuwait
                Author notes
                [*] [* ] Correspondence

                Dr. Mona Al‐Ahmad, Microbiology Department, Faculty of Medicine, Kuwait University, P.O. Box 24923, Safat 13110, Kuwait.

                Email: mona.alahmad@ 123456ku.edu.kw

                Author information
                https://orcid.org/0000-0003-2950-5363
                Article
                JHA2219
                10.1002/jha2.219
                8242544
                34226899
                6faf64db-00b1-4524-aedf-db6cf7fc2851
                © 2021 The Authors. eJHaem published by British Society for Haematology and John Wiley & Sons Ltd.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 April 2021
                : 28 April 2021
                : 04 May 2021
                Page count
                Figures: 0, Tables: 1, Pages: 3, Words: 2055
                Categories
                Case Report
                Case Reports
                Custom metadata
                2.0
                corrected-proof
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.0.2 mode:remove_FC converted:30.06.2021

                covid‐19,thrombocytopenia,ttp,vaccines
                covid‐19, thrombocytopenia, ttp, vaccines

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