Management of Pregnancy-Associated Thrombotic Thrombocytopenia Purpura

Abstract

Thrombotic thrombocytopenia purpura (TTP) is an infrequent but serious disease. Pregnancy is a known risk factor for presentation or relapse of TTP. Difficulties in differentiating TTP from preeclampsia/HELLP (hemolysis, elevated liver enzymes and low platelets) syndrome, and current treatment recommendations are discussed in this case report. A woman with previously treated and stable TTP had a relapse at 36 weeks' gestation. Careful surveillance led to an early diagnosis. Severe disease in the peripartum period was treated successfully with cryosupernatant plasma-based plasmapheresis and platelet transfusion, with good maternal and neonatal outcomes. Cryosupernatant plasma is a viable alternative to fresh frozen plasma for plasmapheresis for TTP and may offer some therapeutic and logistical advantages. Platelet transfusion can be undertaken safely if needed to prevent or treat significant hemorrhage.

Keywords

fresh frozen plasma - cryosupernatant plasma - platelet transfusion - preeclampsia - HELLP syndrome - TTP

• References

• 1 George JN. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354: 1927-1935
• 2 George JN. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood 2010; 116: 4060-4069
• 3 Martin Jr JN, Bailey AP, Rehberg JF, Owens MT, Keiser SD, May WL. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955–2006. Am J Obstet Gynecol 2008; 199: 98-104
• 4 Plaimauer B, Zimmermann K, Völkel D , et al. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood 2002; 100: 3626-3632
• 5 Remuzzi G, Galbusera M, Noris M , et al; Italian Registry of Recurrent and Familial HUS/TTP. Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Blood 2002; 100: 778-785
• 6 Vesely SK, Li X, McMinn JR, Terrell DR, George JN. Pregnancy outcomes after recovery from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Transfusion 2004; 44: 1149-1158
• 7 Rieger M, Mannucci PM, Kremer Hovinga JA , et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood 2005; 106: 1262-1267
• 8 George JN. How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood 2000; 96: 1223-1229
• 9 Scully M, Starke R, Lee R, Mackie I, Machin S, Cohen H. Successful management of pregnancy in women with a history of thrombotic thrombocytopaenic purpura. Blood Coagul Fibrinolysis 2006; 17: 459-463
• 10 Raman R, Yang S, Wu HM, Cataland SR. ADAMTS13 activity and the risk of thrombotic thrombocytopenic purpura relapse in pregnancy. Br J Haematol 2011; 153: 277-279
• 11 Michael M, Elliott EJ, Ridley GF, Hodson EM, Craig JC. Interventions for haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura. Cochrane Database Syst Rev 2009; (1) CD003595
• 12 Lattuada A, Rossi E, Calzarossa C, Candolfi R, Mannucci PM. Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome. Haematologica 2003; 88: 1029-1034
• 13 Molvarec A, Rigó Jr J, Bõze T , et al. Increased plasma von Willebrand factor antigen levels but normal von Willebrand factor cleaving protease (ADAMTS13) activity in preeclampsia. Thromb Haemost 2009; 101: 305-311
• 14 Burrows RF, Kelton JG. Fetal thrombocytopenia and its relation to maternal thrombocytopenia. N Engl J Med 1993; 329: 1463-1466
• 15 Scully M, Longair I, Flynn M, Berryman J, Machin SJ. Cryosupernatant and solvent detergent fresh-frozen plasma (Octaplas) usage at a single centre in acute thrombotic thrombocytopenic purpura. Vox Sang 2007; 93: 154-158
• 16 Fontana S, Kremer Hovinga JA, Lämmle B, Mansouri Taleghani B. Treatment of thrombotic thrombocytopenic purpura. Vox Sang 2006; 90: 245-254
• 17 Nguyen TC, Stegmayr BG, Busund R, Bunchman TE, Carcillo JA. Plasma therapies in thrombotic syndromes. Int J Artif Organs 2005; 28: 459-465
• 18 Altuntas F, Aydogdu I, Kabukcu S , et al. Therapeutic plasma exchange for the treatment of thrombotic thrombocytopenic purpura: a retrospective multicenter study. Transfus Apheresis Sci 2007; 36: 57-67
• 19 Byrnes JJ, Moake JL, Klug P, Periman P. Effectiveness of the cryosupernatant fraction of plasma in the treatment of refractory thrombotic thrombocytopenic purpura. Am J Hematol 1990; 34: 169-174
• 20 Owens MR, Sweeney JD, Tahhan RH, Fortkolt P. Influence of type of exchange fluid on survival in therapeutic apheresis for thrombotic thrombocytopenic purpura. J Clin Apher 1995; 10: 178-182
• 21 Rock G, Shumak KH, Sutton DM, Buskard NA, Nair RC. Members of the Canadian Apheresis Group. Cryosupernatant as replacement fluid for plasma exchange in thrombotic thrombocytopenic purpura. Br J Haematol 1996; 94: 383-386
• 22 Harkness DR, Byrnes JJ, Lian EC, Williams WD, Hensley GT. Hazard of platelet transfusion in thrombotic thrombocytopenic purpura. JAMA 1981; 246: 1931-1933
• 23 Swisher KK, Terrell DR, Vesely SK, Kremer Hovinga JA, Lämmle B, George JN. Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura. Transfusion 2009; 49: 873-887
• 24 Myers TJ, Wakem CJ, Ball ED, Tremont SJ. Thrombotic thrombocytopenic purpura: combined treatment with plasmapheresis and antiplatelet agents. Ann Intern Med 1980; 92 (2 Pt 1) 149-155
• 25 Bobbio-Pallavicini E, Gugliotta L, Centurioni R , et al. Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP. Haematologica 1997; 82: 429-435
• 26 He Y, Chen Y, Zhao Y, Zhang Y, Yang W. Clinical study on five cases of thrombotic thrombocytopenic purpura complicating pregnancy. Aust N Z J Obstet Gynaecol 2010; 50: 519-522