American Journal of Obstetrics & Gynecology
Volume 199, Issue 2 , Pages 98-104 , August 2008

Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006

Presented at the 74th Annual Meeting of the Central Association of Obstetricians and Gynecologists, Chicago, IL, Oct. 17-20, 2007.

  • James N. Martin Jr, MD

      Affiliations

    • Department of Obstetrics and Gynecology (Divisions of Maternal-Fetal Medicine and Women's Health), University of Mississippi Medical Center, Jackson, MS
    • ,
  • Amelia P. Bailey, MD

      Affiliations

    • Department of Obstetrics and Gynecology (Divisions of Maternal-Fetal Medicine and Women's Health), University of Mississippi Medical Center, Jackson, MS
    • ,
  • Jonathan F. Rehberg, MD

      Affiliations

    • Department of Obstetrics and Gynecology (Divisions of Maternal-Fetal Medicine and Women's Health), University of Mississippi Medical Center, Jackson, MS
    • ,
  • Michelle T. Owens, MD

      Affiliations

    • Department of Obstetrics and Gynecology (Divisions of Maternal-Fetal Medicine and Women's Health), University of Mississippi Medical Center, Jackson, MS
    • ,
  • Sharon Dixon Keiser, MD

      Affiliations

    • Department of Obstetrics and Gynecology (Divisions of Maternal-Fetal Medicine and Women's Health), University of Mississippi Medical Center, Jackson, MS
    • ,
  • Warren L. May, PhD

      Affiliations

    • Department of Preventive Medicine, University of Mississippi Medical Center, Jackson, MS.

Received 1 October 2007 ,Revised 28 February 2008 ,Accepted 5 March 2008.

References 

  1. Terrell DR, Williams LA, Vesely SK, Lammie B, Hovinga JA, George JN. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: All patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005;3:1432–1436
  2. George JN. Thrombotic thrombocytopenic purpura. N Engl J Med. 2006;354:1927–1935
  3. Moake JL. Thrombotic microangiopathies: Mechanisms of disease. N Engl J Med. 2002;347:589–600
  4. Moake JL. Thrombotic thrombocytopenic purpura: The systemic clumping “plague”. Annu Rev Med. 2002;53:75–88
  5. George JN. ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. Curr Hem Rep. 2005;4:167–169
  6. Murrin RJA, Murray JA. Thrombotic thrombocytopenic purpura: aetiology, pathophysiology and treatment. Blood Rev. 2006;20:51–60
  7. Nguyen TC, Stegmayr BG, Busund R, Bunchman TE, Carcillo JA. Plasma therapies in thrombotic syndromes. Int J Art Org. 2005;28:459–465
  8. Rehberg JF, Briery CM, Hudson WT, Bofill JA, Martin JN. Thrombotic thrombocytopenic purpura masquerading as hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome in late pregnancy. Obstet Gynecol. 2006;108:817–820
  9. Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol. 2007;109:956–966
  10. Martin JN, Rose CH, Briery CM. Understanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol. 2006;195:914–934
  11. Miner PF, Nutt RL, Thomas ME. Thrombotic thrombocytopenic purpura occurring in pregnancy. Am J Obstet Gynecol. 1955;70:611–617
  12. Reisfield DR. Thrombotic thrombocytopenic purpura and pregnancy. Obstet Gynecol Survey. 1959;14:303–321
  13. O'Leary JA, Marchetti AA. Thrombotic thrombocytopenic purpura in pregnancy. Am J Obstet Gynecol. 1962;83:214–219
  14. Reisfield DR. Death from thrombotic thrombocytopenic purpura during pregnancy. Obstet Gynecol. 1962;19:517–520
  15. Solomon W, Turner DS, Block C, Posner AC. Thrombotic thrombocytopenic purpura in pregnancy. JAMA. 1963;184:587–590
  16. O'Leary JA, Tovell HMM. Thrombotic thrombocytopenic purpura complicating pregnancy. Bulletin Sloane Hosp. 1963;6–9
  17. Castleman BJ, McNeely BU. Case 50-1966—case records of the Massachusetts General Hospital. N Engl J Med. 1966;275:1125–1133
  18. Piver MS, Lisker SA, Rowan N, Weber LL, Brody JI, Beizer LH. Thrombotic thrombocytopenic purpura during pregnancy. Am J Obstet Gynecol. 1968;100:302–304
  19. Hill JB, Cooper WM. Thrombotic thrombocytopenic purpura: treatment with corticosteroids and splenectomy. Arch Intern Med. 1968;122:353–356
  20. Richardson JH, Smith BT. Thrombotic thrombocytopenic purpura: survival in pregnancy with heparin sodium therapy. JAMA. 1968;203:176–177
  21. Tapp E, Geary CG, Dawson DW. Thrombotic micro-angiopathy with macroscopic infarction. J Pathol. 1969;97:711–716
  22. Bernard RP, Bauman AW, Schwartz SI. Splenectomy for thrombotic thrombocytopenic purpura. Ann Surg. 1969;169:616–624
  23. Scharoff JR, Serlin N, Atamer MA. Thrombotic thrombocytopenic purpura: Report of a case treated with splenectomy and steroids. Acta Haematol. 1969;41:180–185
  24. Edmondson WP, Sturgill BC. Clinicopathological conference: Fever, abdominal pain and eclampsia. Va Med Mon (1918). 1970;97:174–185
  25. Mant MJ, Cauchi MN, Medley G. Thrombotic thrombocytopenic purpura: Report of a case with possible immune etiology. Blood. 1972;40:416–421
  26. Moon EC, Kitay DZ. Hematologic problems of pregnancy: II (Thrombotic (thrombohemolytic) thrombocytopenia purpura). J Reprod Med. 1972;9:212–221
  27. Perel ID, Forgan-Smith WR. Thrombotic thrombocytopenic purpura presenting as eclampsia. Aust N Z J Obstet Gynecol. 1972;12:257–262
  28. Jaffe EA, Nachman RL, Merskey C. Thrombotic thrombocytopenic purpura—coagulation parameters in twelve patients. Blood. 1973;42:499–507
  29. Vandewalle A, Kanfer A, Kourilsky O. Oliguric thrombotic microangiopathy during the fifth month of pregnancy. Br Med J. 1975;2:479
  30. Barrett C, Marshall JR. Thrombotic thrombocytopenic purpura. Obstet Gynecol. 1975;46:231–235
  31. Vilanova JR, Norenberg MD, Stuard ID. Thrombotic thrombocytopenic purpura: Systemic embolization from nonbacterial thrombotic endocarditis. N Y State J Med. 1975;75:2246–2248
  32. Dalal FY, Bennett EJ, Grundy EM, Younes S, Orfei E. Anesthetic considerations in diffuse bleeding diathesis of uncertain origin. Anesth Analgesia. 1976;55:173–176
  33. Fuchs WE, George JN, Dotin LN, Sears DA. Thrombotic thrombocytopenic purpura: Occurrence two years apart during late pregnancy in two sisters. JAMA. 1976;235:2126–2127
  34. Neame PB, Hirsh J, Browman G, Denburg J, D'Souza TJ, Gallus A, et al. Thrombotic thrombocytopenic purpura: A syndrome of intravascular platelet consumption. CMAJ. 1976;114:1108–1112
  35. May HV, Harbert GM, Thornton WN. Thrombotic thrombocytopenic purpura associated with pregnancy. Am J Obstet Gynecol. 1976;126:452–458
  36. Fitzgibbons JF, Goodnight SH, Burkhardt JH, Kirk PE. Survival following thrombotic thrombocytopenic purpura in pregnancy. Obstet Gynecol. 1977;50:66s–68s
  37. Bukowski RM, King JW, Hewlett JS. Plasmapheresis in the treatment of thrombotic thrombocytopenic purpura. Blood. 1977;50:413–417
  38. Byrnes JJ, Khurana M. Treatment of thrombotic thrombocytopenic purpura with plasma. N Engl J Med. 1977;297:1386–1389
  39. Lian EC-Y, Harkness DR, Byrnes JJ, Wallach H, Nunez R. Presence of a platelet aggregating factor in the plasma of patients with thrombotic thrombocytopenic purpura (TTP) and its inhibition by normal plasma. Blood. 1979;53:333–338
  40. Yang C, Nussbaum M, Park H. Thrombotic thrombocytopenic purpura in early pregnancy: Remission after plasma exchange. Acta Haematol. 1979;62:112–116
  41. Wurzel JM. TTP lesions in placenta but not fetus. N Engl J Med. 1979;301:503–504
  42. Bateman SM, Hilgard P, Gordon-Smith EC. Thrombotic thrombocytopenic purpura: A possible plasma factor deficiency. Br Soc Haematol. 1979;20:498–499
  43. Perkins RP. Thrombocytopenia in obstetric syndromes: a review. Obstet Gynecol Surv. 1979;34:101–114
  44. Benson DO, Fitzgibbons JF, Goodnight SH. The visual system in thrombotic thrombocytopenic purpura. Ann Ophthalmol. 1980;12:413–417
  45. Walker BK, Ballas SK, Martinez J. Plasma infusion for thrombotic thrombocytopenic purpura during pregnancy. Arch Intern Med. 1980;140:981–983
  46. Cuttner J. Thrombotic thrombocytopenic purpura: A ten-year experience. Blood. 1980;56:302–306
  47. Rossi EC, del Greco F, Kwaan HC, Lerman BB. Hemodialysis-exchange transfusion for treatment of thrombotic thrombocytopenic purpura. JAMA. 1980;244:1466–1468
  48. Shinoda A, Kitada H, Suzuki S, et al. Accessible plasma exchange using membrane filters—a successfully treated case of TTP with repeated plasma exchanges. Artif Organs. 1981;5:248–253
  49. Atlas M, Barkai G, Menczer J, Houlu N, Lieberman P. Thrombotic thrombocytopenic purpura in pregnancy. Br J Obstet Gynaecol. 1982;89:476–479
  50. Kitchens CS. Studies of a patient with recurring thrombotic thrombocytopenic purpura. Am J Hematol. 1982;13:259–267
  51. Holdrinet RSG, DePauw BE, Haanen C. Hormonal dependent thrombotic thrombocytopenic purpura (TTP). Scand J Haematol. 1983;30:250–256
  52. Blakowski SA, Zacharski LR, Telfer MC. Pregnancy complicated by thrombotic thrombocytopenic purpura with fetal sparing. Arch Intern Med. 1983;143:2207–2208
  53. Caggiano V, Fernando LP, Schneider JM, Haesslein HC, Watson-Williams EJ. Thrombotic thrombocytopenic purpura: Report of fourteen cases—occurrence during pregnancy and response to plasma exchange. J Clin Apheresis. 1983;1:71–85
  54. Lian EC-Y, Byrnes JJ, Harkness DR. Two successful pregnancies in a woman with chronic thrombotic thrombocytopenic purpura treated by plasma infusion. Am J Hematol. 1984;16:287–291
  55. Vandekerckhove F, Noens L, Colardyn F, Thiery M, Delbarge W. Thrombotic thrombocytopenic purpura mimicking toxemia of pregnancy. Am J Obstet Gynecol. 1984;150:320–322
  56. Davies GE. Thrombotic thrombocytopenic purpura in pregnancy with maternal survival: Case report. Br J Obstet Gynecol. 1984;91:396–398
  57. Ambrose A, Welham RT, Cefalo RC. Thrombotic thrombocytopenic purpura in early pregnancy. Obstet Gynecol. 1985;66:267–272
  58. Natelson EA, White D. Recurrent thrombotic thrombocytopenic purpura in early pregnancy: effect of uterine evacuation. Obstet Gynecol. 1985;66:54S–56S
  59. Blitzer JB, Granfortuna JM, Gottlieb AJ, et al. Am J Hematol. 1987;24:329–339
  60. Koyama T, Oura Y, Kakishita E, et al. A case report: Successful delivery in a female with thrombotic thrombocytopenic purpura. Japan J Med. 1987;26:381–384
  61. Pinette MG, Vintzileos AM, Ingardia CJ. Thrombotic thrombocytopenic purpura as a cause of thrombocytopenia in pregnancy: Literature review. Am J Perinatol. 1989;6:55–57
  62. Ezra Y, Mordel N, Sadovsky E, Schenker JG, Eldor A. Successful pregnancies of two patients with relapsing thrombotic thrombocytopenic purpura. Int J Gynecol Obstet. 1989;29:359–363
  63. Koyama T, Suehiro A, Kakishita E, et al. Efficacy of high molecular weight fraction of plasma for the maintenance of pregnancy associated with thrombotic thrombocytopenic purpura. Am J Hematol. 1990;35:179–183
  64. Maina A, Donvito V, Giachino O, Stratta P, Camaschella C. Thrombotic thrombocytopenic purpura in pregnancy with maternal and fetal survival: Case report. Br J Obstet Gynaecol. 1990;97:443–445
  65. Roberts AW, Gillett EA, Fleming SJ. Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura: Outcome with plasma exchange. J Clin Apheresis. 1991;6:150–154
  66. Thorp JM, Wells SR, Bowes WA. The obfuscation continues: Severe preeclampsia versus thrombotic thrombocytopenic purpura. N C Med J. 1991;52:126–128
  67. Khoo US, Dickens P, Cheung ANY. Rapid death from thrombotic thrombocytopenic purpura following cesarean section. Forensic Sci Int. 1992;54:75–80
  68. Permezel M, Lee N, Corry J. Thrombotic thrombocytopenic purpura in pregnancy. Aust N Z J Obstet Gynaecol. 1992;32:278–280
  69. Wiznitzer A, Mazor M, Leiberman JR, Var-Levie Y, Gurman G, Glezerman M. Familial occurrence of thrombotic thrombocytopenic purpura in two sisters during pregnancy. Am J Obstet Gynecol. 1992;166:20–21
  70. Uslu M, Guzelmeric K, Asut I. Familial thrombotic thrombocytopenic purpura imitating HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) in two sisters during pregnancy. Am J Obstet Gynecol. 1994;699–700
  71. Alqadah F, Zebeib MA, Awidi AS. Thrombotic thrombocytopenic purpura associated with pregnancy in two sisters. Postgrad Med J. 1993;69:229–231
  72. Rozdzinski E, Hertenstein B, Schmeiser T, Seifried E, Kurrle E, Heimpel H. Thrombotic thrombocytopenic purpura in early pregnancy with maternal and fetal survival: Case report. Ann Hematol. 1992;64:245–248
  73. Vianelli N, Gugliotta , Catani L, Baravelli S, Tura S. Thrombotic thrombocytopenic purpura: relapse and pregnancy. Haematologica. 1993;78:259
  74. Helou J, Nakhle S, Shoenfeld S, Nasseir T, Shalev E. Postpartum thrombotic thrombocytopenic purpura: Report of a case and review of the literature. Obstet Gynecol Surv. 1994;49:785–789
  75. Olenich M, Schattner E. Postpartum thrombotic thrombocytopenic purpura (TTP) complicating pregnancy-associated immune thrombocytopenic purpura (ITP). Ann Intern Med. 1994;120:845–847
  76. Hayward CPM, Sutton DMC, Carter WH, et al. Treatment outcomes in patients with adult thrombotic thrombocytopenic purpura—hemolytic uremic syndrome. Arch Intern Med. 1994;154:982–987
  77. Mokrzycki MH, Rickles RF, Kaplan AA, Kohn OF. Thrombotic thrombocytopenic purpura in pregnancy: Successful treatment with plasma exchange. Blood Purif. 1995;13:271–282
  78. Puza S, Malee MP. Chronic relapsing thrombotic thrombocytopenic purpura in pregnancy: A case report. J Matern Fetal Med. 1996;5:328–332
  79. Egerman RS, Witlin AG, Friedman SA, Sibai BM. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in pregnancy: Review of 11 cases. Am J Obstet Gynecol. 1996;175:950–956
  80. Ezra Y, Rose M, Eldor A. Therapy and prevention of thrombotic thrombocytopenic purpura during pregnancy: A clinical study of 16 pregnancies. Am J Hematol. 1996;51:1–6
  81. Rund D, Schaap T, Gillis S. Intensive plasmapheresis for severe thrombotic thrombocytopenic purpura: long-term clinical outcome. J Clin Apheresis. 1997;12:194–195
  82. Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lammie B. Deficient activity of von Willebrand Factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997;89:3097–3103
  83. Dashe JS, Ramin SM, Cunningham FG. The long-term consequences of thrombotic microangiopathy (thrombotic thrombocytopenic purpura and hemolytic uremic syndrome) in pregnancy. Obstet Gynecol. 1998;91:662–668
  84. Kemp WL, Barnard JJ, Prahlow JA. Death due to thrombotic thrombocytopenic purpura in pregnancy. Am J Forensic Med Pathol. 1999;20:189–198
  85. Brostrom S, Bergmann OJ. Thrombotic thrombocytopenic purpura: A difficult differential diagnosis in pregnancy. Acta Obstet Gynecol Scand. 2000;79:84–85
  86. Mastrobattista JM, Ramin SM, Gilstrap LC. Thrombotic thrombocytopenic purpura in pregnancy. Prim Care Update Ob Gyns. 2000;7:168–171
  87. Cao L, Morrow M, lally T, Lin E, deChristopher PJ. Surge of anti-SSA antibody associated with fulminant thrombotic thrombocytopenic purpura in pregnancy. South Med J. 2001;94:1219–1222
  88. Ranzini AC, Chavez MR, Ghigliotty B, Porcelli M. Thrombotic thrombocytopenic purpura and human immunodeficiency virus complicating pregnancy. Obstet Gynecol. 2002;100:1133–1136
  89. Proia A, Paesano R, Torcia F, et al. Thrombotic thrombocytopenic purpura and pregnancy: A case report and a review of the literature. Ann Hematol. 2002;81:210–214
  90. Cosmai EM, Puzis L, Tsai H-M, Lian EC-Y. Thrombotic thrombocytopenic purpura and cardiomyopathy in pregnancy reversed by combined plasma exchange and infusion. Eur J Haematol. 2002;68:239–242
  91. Obeidat B, MacDougall J, Harding K. Plasma exchange in a woman with thrombotic thrombocytopenic purpura or severe preeclampsia. Br J Obstet Gynaecol. 2002;109:961–962
  92. Sivakumaran M, Roland J. Prophylactic treatment with fresh frozen plasma in chronic thrombotic thrombocytopenic purpura. Br J Haemat. 2002;117:480–483
  93. Richmond JR, Koufogianis V, Benjamin A, Warner MN. A case of thrombotic thrombocytopenic purpura and neonatal alloimmune thrombocytopenia in the same pregnancy. Br J Obstet Gynaecol. 2003;110:533–536
  94. Ducloy-Bouthors A-S, Caron C, Subtil D, et al. Thrombotic thrombocytopenic purpura: Medical and biological monitoring of six pregnancies. Eur J Obstet Gynecol Reprod Biol. 2003;111:146–152
  95. Patnaik MM, Deshpande AK, Nagar VS, Algotar KM. Thrombotic microangiopathies presenting as an obstetric emergency. J Assoc Physicians India. 2004;52:152–153
  96. Shamseddine A, Chehal A, Usta I, Salem Z, El-Saghir N, Taher A. Thrombotic thrombocytopenic purpura and pregnancy: Report of four cases and literature review. J Clin Apheresis. 2004;19:5–10
  97. Soltes L, Schmalfuss IM, Bhatti MT. Cortical blindness due to reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura and preeclampsia. Arch Opthal. 2004;122:1885–1887
  98. Sherer DM, Sanmugarajah J, Dalloul M, Temkin SM, Thanus J, Abulafia O. Thrombotic thrombocytopenic purpura in a patient with acquired immunodeficiency syndrome at 28 weeks gestation. Am J Perinatol. 2005;22:223–225
  99. 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
  100. Castella M, Pujol M, Julia A, et al. Thrombotic thrombocytopenic purpura and pregnancy: A review of ten cases. Vox Sang. 2004;87:287–290
  101. Scully M, Starke R, Lee R, et al. Successful management of pregnancy in women with a history of thrombotic thrombocytopenic purpura. Blood Coagul Fibrinolysis. 2006;17:459–463
  102. Moake JL. Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome. Arch Pathol Lab Med. 2002;126:1430–1433
  103. Simas TAM, Crawford SL, Solitro MJ, Frost SC, Meyer BA, Maynard SE. Angiogenic factors for the prediction of preeclampsia in high risk women. Am J Obstet Gynecol. 2007;197:244–247
  104. Salahuddin S, Lee Y, Vadnais M, Sachs BP, Karumanchi A, Lim K-H. Diagnostic utility of soluble fms-like tyrosine kinase 1 and soluble endoglin in hypertensive diseases of pregnancy. Am J Obstet Gynecol. 2007;197:28–29
  105. Staff AC, Braekke K, Johnsen GM, Karumachi A, Harsem NK. Circulating concentrations of soluble endoglin (CD105) in fetal and maternal serum and in amniotic fluid in preeclampsia. Am J Obstet Gynecol. 2007;197:176–178
  106. Robinson CJ, Johnson DD. Soluble endoglin as a second trimester marker for preeclampsia. Am J Obstet Gynecol. 2007;197:174–176
  107. Allford SL, Hunt BJ, Rose P, Machin SJ. Guideline: Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Hematol. 2003;120:556–573
  108. Barbot J, Costa E, Guerra M, Barreirinho MS, Isvarlal P, Robles R. Ten years of prophylactic treatment with fresh frozen plasma in a child with chronic relapsing TTP as a result of a congenital deficiency of von Willebrand factor cleaving protease. Br J Haematol. 2001;113:649–651
  109. McCarthy LJ, Dlott JS, Orzi A, Waxman D, Miraglia CC, Danielson CFM. Thrombotic thrombocytopenic purpura: Yesterday, today, tomorrow. Ther Apher Dial. 2004;8:80–86
  110. Sullivan CA, Martin JN. Thrombotic microangiopathies. In:  Didly GA editors. Critical care obstetrics. 4th ed.. Maiden (MA): Blackwell Publishing; 2004;p. 408–419
  111. Lattuada A, Rossi E, Calzarossa C, Candolfi R, Mannucci PM. Mild to moderate reduction of von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome. Haematologica. 2003;88:1029–1034
  112. Hulstein JJJ, Van Runnard Heimel PJ, Franx A, et al. Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. J Thromb Haemost. 2006;4:2569–2575
  113. Nagajothi N, Braverman A. Elevated red cell distribution width in the diagnosis of TTP patients presenting with anemia and thrombocytopenia. South Med J. 2007;100:257–259
  114. George JN. The association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Curr Opin Hematol. 2003;10:339–344

 Reprints not available from the authors.

PII: S0002-9378(08)00272-X

doi: 10.1016/j.ajog.2008.03.011

American Journal of Obstetrics & Gynecology
Volume 199, Issue 2 , Pages 98-104 , August 2008

Article Tools