Emergency Plasmapheresis in a case of ThromboticThrombocytopenic Purpura (TTP)

Mariaserena Pioli Di Marco, Francesco Guercini, Chiara Busti, Olivia Minelli

DOI: https://doi.org/10.7175/cmi.v7i3.669


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).


Plasmapheresis; ThromboticThrombocytopenic Purpura (TTP); Emergency

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  • 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


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