Summary
Thrombotic thrombocytopenic purpura (TTP) is a hematologic disorder which is clinically characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurologic symptoms, and cardiac and renal involvement. The pathogenic mechanisms of this disease are poorly understood. It is well known that TTP is associated with pregnancy and that prognosis for the mother and child is poor. We present the first case of a severe TTP diagnosed in the first trimester of pregnancy (13th week of gestation) with maternal survival and birth of a healthy child which required continuous and intensive treatment with plasmatherapy until delivery. During a period of 24 weeks several attempts to discontinue plasma therapy failed because of continuous active disease, and it became evident that plasma infusions were not as effective as plasma exchanges. The fact that the patient entered into remission soon after delivery of a healthy child by cesarean section in the 37th gestational week shows that in this case pregnancy activated an unknown factor which does not cross the placenta and which can be removed by plasmapheresis.
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References
Amorosi EL, Ultmann JE (1966) Thrombotic thrombocytopenic purpura: report of 16 cases and review of literature. Medicine (Baltimore) 45: 139–159
Blakowski SA, Zacharski LR, Telfer MC (1983) Pregnancy complicated by thrombotic thrombocytopenic purpura with fetal sparing. Arch Intern Med 143: 2207–2208
Caggiano V, Fernando LP, Schneider JM, Haesslein HC, Watson-Williams EJ (1983) Thrombotic thrombocytopenic purpura: report of fourteen cases — occurrence during pregnancy and response to plasma exchange. J Clin Apheresis 1: 71–85
Davies GE (1984) Thrombotic thrombocytopenic purpura in pregnancy with maternal survival. Case report. Br J Obstet Gynaecol 91: 396–398
Dixit R, Krieg AM, Atkinson JP (1985) Thrombotic thrombocytopenic purpura developing during pregnancy in ac2-deficient patient with a history of systemic lupus erythematosus. Arthritis Rheum 28: 341–344
Glas-Greenwalt P, Kont KS, Pollak VE, et al. (1986) Severely depressed fibrinolysis in 12 patients with thrombotic thrombocytopenic purpura. J Lab Clin Med 108: 415–456
Hill JB, Cooper WM (1968) Thrombotic thrombocytopenic purpura. Treatment with corticosteroids and splenectomy. Arch Intern Med 122: 353–356
Kelton JG, Moore J, Santos A, Sheridan D (1984) The detection of a platelet-agglutinating factor in thrombotic thrombocytopenic purpura. Ann Intern Med 101: 589–593
Koyama T, Kakishita E, Nagai K (1989) Factors affecting the response to plasma therapy in thrombotic thrombocytopenic purpura. Jpn J Med 28: 16–19
Lee SH, Wainscoat JS, Zeitlin H, Bolton FG, Leaver HA, Seawright A, Preece JM (1981) Prostacyclin and thromboxane A2 in thrombotic thrombocytopenic purpura. Br Med J [Clin Res] 283: 1351–1352
Lian EC (1987) Pathogenesis of thrombotic thrombocytopenic purpura. Semin Hematol 24: 82–100
Lian ECY, Harkness DR, Byrnes JJ, et al. (1979) The presence of a platelet-aggregating factor in the plasma of patients with thrombotic thrombocytopenic purpura and its inhibition by normal plasma. Blood 53: 333–338
Lian ECY, Byrnes JJ, Harkness DR (1984) Two successful pregnancies in a woman with chronic thrombocytopenic purpura treated by plasma infusions. Am J Hematol 16: 287–292
Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL, Deykin D (1982) Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 307: 1432–1435
Monteagudo J, Pereira A, Reverler JC, Pijoan J, Tusell J, Puig L, Ordinas A, Castillo R (1991) Thrombin generation and fibrinolysis in the thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. Thromb Haemost 66: 515–628
Moschcowitz E (1925) Acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. An undescribed disease. Am J Clin Pathol 36: 89–93
Neame PB (1973) Disseminated intravascular platelet aggregation in thrombotic thrombocytopenic purpura. Lancet 1: 1320–1321
Pinette MG, Vintzileos AM, Ingardia CJ (1989) Thrombotic thrombocytopenic purpura as a cause of thrombocytopenia in pregnancy: literature review. Am J Perinatol 6: 55–57
Remuzzi G, Imberti L, de Gaetano G (1981) Prostacyclin deficiency in thrombotic microangiopathy (letter). Lancet 2: 1422–1423
Ridolfi RL, Bell WR (1981) Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature. Medicine (Baltimore) 60: 413–428
Ruggenenti P, Remuzzi G (1990) Thrombotic thrombocytopenic purpura and related disorders. Hematol Oncol Clin North Am 4: 219–241
Staiger HJ, Fettig O, Theml H (1989) Thrombotisch-thrombozytopenische Purpura (Moschcowitz-Syndrome) bei einer Patientin mit EPH-Gestose (letter). Geburtshilfe Frauenheilkd 49: 1099–1100
Taft EG (1990) Advances in the treatment of TTP. Apheresis 151–155
Upshaw JD, Reidy TJ, Groshart K (1985) Thrombotic thrombocytopenic purpura in pregnancy: response to plasma manipulations. South Med J 78: 677–680
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Rozdzinski, E., Hertenstein, B., Schmeiser, T. et al. Thrombotic thrombocytopenic purpura in early pregnancy with maternal and fetal survival. Ann Hematol 64, 245–248 (1992). https://doi.org/10.1007/BF01738304
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DOI: https://doi.org/10.1007/BF01738304