Korean J Obstet Gynecol.  2010 May;53(5):434-442. 10.5468/kjog.2010.53.5.434.

Thrombotic thrombocytopenic purpura in three pregnancies

• Yoon WS
• Lee JW
• Kim YH
• Ahn HY
• Shin JC

• KMID: 2273876
• DOI: http://doi.org/10.5468/kjog.2010.53.5.434

Abstract

Thromobotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction, is severe multisystem disorder. TTP-HUS occurs predominantly in the reproductive aged-women, associated with poor prognosis. Although the morbidity and mortality have been significantly decreased by using plasma exchange therapy, refractory TTP-HUS remains a tremendous problem. It is crucial to differentiate other microangiopathic hemolytic anemia disease with a confusing presentation and to perform the immediate plasmapheresis. We have experienced three cases, which were initially diagnosed as HELLP syndrome or immune thrombocytopenic purpura. Despite of aggressive plasmapheresis, two women died. We present these cases with a review of the literature on pregnancy-associated thrombotic microangiopathy, including ADAMTS-13 activity assay as a new diagnostic test.

Keyword

Thrombotic thrombocytopenic purpura; HELLP Syndrome; Pre-Eclampsia; ADAMTS-13; Pregnancy complications; Hematologic/pathology/therapy

MeSH Terms

Anemia, Hemolytic
Central Nervous System
Diagnostic Tests, Routine
Female
Fever
HELLP Syndrome
Humans
Plasma Exchange
Plasmapheresis
Pre-Eclampsia
Pregnancy
Pregnancy Complications
Prognosis
Purpura, Thrombocytopenic, Idiopathic
Purpura, Thrombotic Thrombocytopenic
Thrombocytopenia
Thrombotic Microangiopathies

Figure

• 

  Fig. 1 Schistocytes are scattered in peripheral blood smear.

• 

  Fig. 2 Abdominal CT shows massive ascites (black arrow), acute pancreatitis (white arrow) and infrarenal inferior vena cava thrombosis (dotted arrow).

Reference

1. Stella CL, Dacus J, Guzman E, Dhillon P, Coppage K, How H, et al. The diagnostic dilemma of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in the obstetric triage and emergency department: lessons from 4 tertiary hospitals. Am J Obstet Gynecol. 2009. 200:381 e1–381 e6.

2. McCrae KR. Thrombocytopenia in pregnancy: differential diagnosis, pathogenesis, and management. Blood Rev. 2003. 17:7–14.

3. Martin JN Jr, 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. Mastrobattista JM, Ramin SM, Gilstrap LC. Thrombotic thrombocytopenic purpura in pregnancy. Prim Care Update Ob Gyns. 2000. 7:168–171.

5. Miller DP, Kaye JA, Shea K, Ziyadeh N, Cali C, Black C, et al. Incidence of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Epidemiology. 2004. 15:208–215.

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. 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.

8. George JN. How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood. 2000. 96:1223–1229.

9. Moake JL. von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura. Semin Hematol. 2004. 41:4–14.

10. Moake J. Thrombotic thrombocytopenia purpura (TTP) and other thrombotic microangiopathies. Best Pract Res Clin Haematol. 2009. 22:567–576.

11. 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 Apher. 2004. 19:5–10.

12. 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.

13. Crowther MA, George JN. Thrombotic thrombocytopenic purpura: 2008 update. Cleve Clin J Med. 2008. 75:369–375.

14. Jang MJ, Oh SY, Chong SY, Kang MS, Oh D. The usefulness of the new ADAMTS-13 activity assay using a fluorescence-quenching substrate for the diagnosis of thrombotic thrombocytopenic purpura. Korean J Hematol. 2005. 40:226–230.

15. Ilter E, Haliloglu B, Temelli F, Ozden S. Thrombotic thrombocytopenic purpura and pregnancy treated with fresh-frozen plasma infusions after plasmapheresis: a case report. Blood Coagul Fibrinolysis. 2007. 18:689–690.

16. Lee YJ, Shim H, Park DE. A Splenectomy in plasma exchange refractory thrombotic thrombocytopenia purpura. Korean J Lab Med. 2006. 26:119–122.

17. D'Angelo A, Fattorini A, Crippa L. Thrombotic microangiopathy in pregnancy. Thromb Res. 2009. 123:Suppl 2. S56–S62.