Emergency Plasmapheresis in a case of ThromboticThrombocytopenic Purpura (TTP)
DOI:
https://doi.org/10.7175/cmi.v7i3.669Keywords:
Plasmapheresis, ThromboticThrombocytopenic Purpura (TTP), EmergencyAbstract
An 84 year-old female was admitted to our Department of Vascular Internal Medicine after a sudden onset of weakness on her right side and aphasia along with signs of myocardial ischemia from Electrocardiogram (EKG). Clinical and blood exams led to a suspicion of Moschcowitz syndrome, which was reinforced by the presence of numerous schistocytes on a peripheral blood smear.
Due to a rapid deterioration of vital signs as well as alertness, the patient underwent an emergency transfusion and plasmapheresis treatment as recommended by American Society of Apheresis (ASFA) guidelines: one plasma volume was replaced with fresh frozen plasma (FFP) every 24 hours, for the first eight days, in order to reach at least a level of 150,000 platelets/mm3 over three consecutive days accompanied by a decrease in LDH until to 670 UI/l.
After this therapy, the clinical picture significantly improved with a complete recovery of consciousness and the disappearance of neurological defects.
Examinations to determine the etiology made us hypothesize a secondary status of thrombotic thrombocytopenic purpura due to an autoimmune disorder compatible with Sjogren’s syndrome. The patient was discharged and prescribed prednisone.
Currently the patient is in good clinical condition and continues the therapy with prednisone (5 mg/die).
References
Tsai HM. Advances in the pathogenesis, diagnosis and treatment of thrombotic thrombocytopenic purpura. J Am Soc Nephrol 2003; 14: 1072-81; http://dx.doi.org/10.1097/01.ASN.0000060805.04118.4C
Sadler JE, Moake JL, Miyata T, et al. Recent advances in thrombotic thrombocytopenic purpura. Hematology (Am Soc Hematol Educ Program) 2004: 407-23; http://dx.doi.org/10.1182/asheducation-2004.1.407
Matsumoto M, Yagi H, Ishizashi H, et al. The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic syndrome. Semin Hematol 2004; 41: 68-74; http://dx.doi.org/10.1053/j.seminhematol.2003.10.009
Schulman I, Pierce M, Lukens A, et al. Studies on thrombopoeisis. A factor in normal human plasma required for platelet production; chronic thrombocytopenia due to its deficiency. Blood 1960; 16: 943-57
Upshaw JD. Congenital deficiency of a factor in normal plasma that reverse microangiopathic hemolysis and thrombocytopenia. N Eng J Med 1978; 298: 1350-2; http://dx.doi.org/10.1056/NEJM197806152982407
Levy GG, Nichols WC, Liane EC, et al. Mutation in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001; 413: 488-94; http://dx.doi.org/10.1038/35097008
Furlan M, Robles R, Galbusera M, et al. Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Eng J Med 1998; 339: 1578-84; http://dx.doi.org/10.1056/NEJM199811263392202
Tsai HM, Lian E. Antibodies to von Willebrand factor cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339:1585-94; http://dx.doi.org/10.1056/NEJM199811263392203
Klaus C, Plaimauer B, Studt JD, et al. Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic Thrombocytopenic purpura. Blood 2004; 103: 4514-9; http://dx.doi.org/10.1182/blood-2003-12-4165
Scheiflinger F, Knöbl P, Trattner B, et al. Nonneutralizing IgM and IgG antibodies to von Willebrand factor cleaving protease (ADAMTS 13) in a patient with thrombotic thrombocytopenic purpura. Blood 2003; 102: 3241-3; http://dx.doi.org/10.1182/blood-2003-05-1616
Rieger M, Mannucci PM, Hovinga JA, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated disease. Blood 2005; 106:1262-67; http://dx.doi.org/10.1182/blood-2004-11-4490
van der Plas RM, Schiphorst ME, Huizinga EG, et al. von Willebrand factor proteolysis is deficient in classic but not in bone marrow transplantation associated, thrombotic thrombocytopenic purpura. Blood 1999; 93:3798-802.
Mannucci PM, Lavoretano S, Peyvandi F. The thrombotic microangiopathies. Blood Transfus 2005; 3: 120-35
Schwartz J, Winters JL, Padmanabhan A. Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the writing commitee of the American Society for Apheresis: The six special issue. J Clin Apher 2013; 28:145-284; http://dx.doi.org/10.1002/jca.21276
George JN. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood 2010; 116: 4060-69; http://dx.doi.org/10.1182/blood-2010-07-271445
Amorosi EL, Ultmann JE. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine 1966; 45: 139-59; http://dx.doi.org/10.1097/00005792-196603000-00003
Ridolfi RL, Bell WR. Thrombotic thrombocytopenic purpura: report of 25 cases and review of the literature. Medicine 1981; 60: 413-28; http://dx.doi.org/10.1097/00005792-198111000-00003
Rose M, Eldor A. High incidence of relapses in thrombotic thrombocytopenic purpura. Am J Med 1987; 83: 437-44; http://dx.doi.org/10.1016/0002-9343(87)90753-4
Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 1991; 325: 393-7; http://dx.doi.org/10.1056/NEJM199108083250604
Altuntas F, Aydogdu I, Kabukcu S. Therapeutic plasma exchange for the treatment of thrombotic thrombocytopenic purpura: a retrospective multicenter study. Transfus Apher Scie 2007; 36: 57-77; http://dx.doi.org/10.1016/j.transci.2006.05.014
Vesely SK, George JN, Lämmle B, et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 2003; 102: 60-8; http://dx.doi.org/10.1182/blood-2003-01-0193
Published
Issue
Section
License
Authors who publish with this journal agree to the following terms:- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution Non-Commercial 4.0 Licence that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal. The Publication Agreement can be downloaded here, and should be signed by the Authors and sent to the Publisher when the article has been accepted for publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access).
- Authors are permitted to post their work online after publication (the article must link to publisher version, in html format)