Maternal Death Due to Refractory Thrombotic Thrombocytopenic Purpura (TTP) Associated With Superimposed Preeclampsia, Sepsis, and Intracranial Hemorrhage: A Case Report
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Abstract
Thrombotic Thrombocytopenic Purpura (TTP) is an acute, rare, potentially life-threatening disorder presenting with thrombocytopenia, hemolytic anemia, and clinical consequences of microvascular thrombosis caused by a deficiency of ADAMS13 [1, 2]. The incidence of acquired TTP is approximately three cases per one million adults per year, based on the Oklahoma TTP-Hemolytic uremic syndrome (HUS) Registry [3]. Here, we present a case of TTP with persistent severely deficient ADAMS13 activity and recurrent relapses. She had a refractory relapse in the third trimester of pregnancy, complicated with superimposed severe preeclampsia and sepsis, and finally expired due to intracranial hemorrhage (ICH).
[1] Fujkawa K, Suzuki H, McMullen B, Chung D. Purification on hu- man von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001; 98(6):1662-6. [DOI:10.1182/blood.V98.6.1662] [PMID]
[2] Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, et al. Mutation in membe of the ADAMS gene family cause throm- botic thrombocytopenic purpura. Nature. 2001; 413(6855):488-49. [DOI:10.1038/35097008] [PMID]
[3] Reese JA, Muthurajah DS, Kremer Hovinga JA, Vesely SK, Terrell DR, George JN. Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired ADAMS13 deficiency: Com- parison of incidence, demographic and clinical features. Pediatr Blood Cancer. 2013; 60(10):1676-82. [DOI:10.1002/pbc.24612] [PMID]
[4] Scully M, Thomas M, Underwood M, Watson H, Langley K, Camilleri RS, et al. Thrombotic thrombocytopenic purpura and pregnancy: Pres- entation, management, and subsequent pregnancy outcomes. Blood. 2014; 124(2):211-9. [DOI:10.1182/blood-2014-02-553131] [PMID]
[5] George JN. How I treat patients with thrombotic thrombocyto- penic purpura: 2010. Blood. 2010; 116(20):4060-9. [DOI:10.1182/ blood-2010-07-271445] [PMID]
[6] Crawley JT, Scully MA. Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies. Hematology Am Soc Hematol Educ Program. 2013; 2013:292-9. [DOI:10.1182/ asheducation-2013.1.292] [PMID]
[7] Nokes T, George JN, Vesely SK, Awab A. Pulmonary involvement in patients with thrombotic thrombocytopenic purpura. Euro- pean Journal of Haematology. 2014; 92(2):156-63. [DOI:10.1111/ ejh.12222] [PMID]
[8] Réti M, Farkas P, Csuka D, Rázsó K, Schlammadinger Á, Udvardy ML, et al. Complement activation in thrombotic thrombocytopenic pur- pura. Journal of Thrombosis and Haemostasis. 2012; 10(5):791-8. [DOI:10.1111/j.1538-7836.2012.04674.x] [PMID]
[9] Gernsheimer T, James AH, Stasi R. How I treat thrombocyto- penia in pregnancy. Blood. 2013; 121(1):38-47. [DOI:10.1182/ blood-2012-08-448944] [PMID]
[10] Battinelli EM. TTP and pregnancy. Blood. 2014; 123(11):1624-5. [DOI:10.1182/blood-2014-01-549469] [PMID]
[11] Vesely SK, Li X, McMinn JR, Terrell DR, George JN. Pregnancy out- comes after recovery from thrombotic thrombocytopenic purpura- hemolytic uremic syndrome. Transfusion. 2004; 44(8):1149-58. [DOI:10.1111/j.1537-2995.2004.03422.x] [PMID]
[12] Scully M, Starke R, Lee R, Mackie I, Machin S, Cohen H. Successful management of pregnancy in women with a history of thrombotic thrombocytopenic purpura. Blood Coagulation & Fibrinolysis. 2006; 17(6):459-63. [DOI:10.1097/01.mbc.0000240918.65306.20] [PMID]
[13] Raman R, Yang S, Wu HM, Cataland SR. ADAMDS13 activity and the risk of thrombotic thrombocytopenic purpura relapse in pregnancy. British Journal of Haematology. 2011; 153(2):277-279. [DOI:10.1111/j.1365-2141.2010.08558.x] [PMID]
[14] Jiang Y, McIntosh JJ, Reese JA, Deford CC, Kremer Hovinga JA, Lämmle B, et al. Pregnancy outcomes following recovery from acquired throm- botic thrombocytopenic purpura. Blood. 2014; 123(11):1674-80. [DOI:10.1182/blood-2013-11-538900] [PMID] [PMCID]
[15] George JN. Clinical practice. Thrombotic thrombocytopenic pur- pura. The New England Journal of Medicine. 2006; 354(18):1927-35. [DOI:10.1056/NEJMcp053024] [PMID]
[16] Martin JN Jr, Bailey AP, Rehberg JF, Owens MT, Keiser SD, May WL. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006. American Journal of Obstetrics and Gynecology. 2008; 199(2):98-104. [DOI:10.1016/j.ajog.2008.03.011] [PMID]
[17] Lattuada A, Rossi E, Calzarossa C, Candolfi R, Mannucci PM. Mild to moderate reduction of a Willebrand factor cleaving protease (ADAMS13) in pregnant women with HELLP microangiopathic syn- drome. Haematologica. 2003; 88(9):1029-34. [PMID]
[18] Molvarec A, Rigó J Jr, Bõze T, Derzsy Z, Cervenak L, Makó V, et al. Increased plasma von Willebrand factor cleaving protease (AD- AMS13) activity in preeclampsia. Thrombosis and Haemostasis. 2009; 101(2):305-11. [DOI:10.1160/TH08-05-0330] [PMID]
[19] Sayani FA, Abrams CS. How I treat refractory thrombotic throm- bocytopenic purpura. Blood. 2015; 125(25):3860-7. [DOI:10.1182/ blood-2014-11-551580] [PMID] [PMCID]
[20] Scully M, McDonald V, Cavenagh J, Hunt BJ, Longair I, Cohen H, et al. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombo- cytopenic purpura. Blood. 2011; 118(7):1746-53. [DOI:10.1182/ blood-2011-03-341131] [PMID]
[21] Chaturvedi S, Abbas H, McCrae KR. Increased morbidity during long-term follow-up of survivors of thrombotic thrombocytopenic purpura. American Journal of Hematology. 2015; 90(10):E208. [DOI:10.1002/ajh.24138] [PMID] [PMCID]
[2] Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, et al. Mutation in membe of the ADAMS gene family cause throm- botic thrombocytopenic purpura. Nature. 2001; 413(6855):488-49. [DOI:10.1038/35097008] [PMID]
[3] Reese JA, Muthurajah DS, Kremer Hovinga JA, Vesely SK, Terrell DR, George JN. Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired ADAMS13 deficiency: Com- parison of incidence, demographic and clinical features. Pediatr Blood Cancer. 2013; 60(10):1676-82. [DOI:10.1002/pbc.24612] [PMID]
[4] Scully M, Thomas M, Underwood M, Watson H, Langley K, Camilleri RS, et al. Thrombotic thrombocytopenic purpura and pregnancy: Pres- entation, management, and subsequent pregnancy outcomes. Blood. 2014; 124(2):211-9. [DOI:10.1182/blood-2014-02-553131] [PMID]
[5] George JN. How I treat patients with thrombotic thrombocyto- penic purpura: 2010. Blood. 2010; 116(20):4060-9. [DOI:10.1182/ blood-2010-07-271445] [PMID]
[6] Crawley JT, Scully MA. Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies. Hematology Am Soc Hematol Educ Program. 2013; 2013:292-9. [DOI:10.1182/ asheducation-2013.1.292] [PMID]
[7] Nokes T, George JN, Vesely SK, Awab A. Pulmonary involvement in patients with thrombotic thrombocytopenic purpura. Euro- pean Journal of Haematology. 2014; 92(2):156-63. [DOI:10.1111/ ejh.12222] [PMID]
[8] Réti M, Farkas P, Csuka D, Rázsó K, Schlammadinger Á, Udvardy ML, et al. Complement activation in thrombotic thrombocytopenic pur- pura. Journal of Thrombosis and Haemostasis. 2012; 10(5):791-8. [DOI:10.1111/j.1538-7836.2012.04674.x] [PMID]
[9] Gernsheimer T, James AH, Stasi R. How I treat thrombocyto- penia in pregnancy. Blood. 2013; 121(1):38-47. [DOI:10.1182/ blood-2012-08-448944] [PMID]
[10] Battinelli EM. TTP and pregnancy. Blood. 2014; 123(11):1624-5. [DOI:10.1182/blood-2014-01-549469] [PMID]
[11] Vesely SK, Li X, McMinn JR, Terrell DR, George JN. Pregnancy out- comes after recovery from thrombotic thrombocytopenic purpura- hemolytic uremic syndrome. Transfusion. 2004; 44(8):1149-58. [DOI:10.1111/j.1537-2995.2004.03422.x] [PMID]
[12] Scully M, Starke R, Lee R, Mackie I, Machin S, Cohen H. Successful management of pregnancy in women with a history of thrombotic thrombocytopenic purpura. Blood Coagulation & Fibrinolysis. 2006; 17(6):459-63. [DOI:10.1097/01.mbc.0000240918.65306.20] [PMID]
[13] Raman R, Yang S, Wu HM, Cataland SR. ADAMDS13 activity and the risk of thrombotic thrombocytopenic purpura relapse in pregnancy. British Journal of Haematology. 2011; 153(2):277-279. [DOI:10.1111/j.1365-2141.2010.08558.x] [PMID]
[14] Jiang Y, McIntosh JJ, Reese JA, Deford CC, Kremer Hovinga JA, Lämmle B, et al. Pregnancy outcomes following recovery from acquired throm- botic thrombocytopenic purpura. Blood. 2014; 123(11):1674-80. [DOI:10.1182/blood-2013-11-538900] [PMID] [PMCID]
[15] George JN. Clinical practice. Thrombotic thrombocytopenic pur- pura. The New England Journal of Medicine. 2006; 354(18):1927-35. [DOI:10.1056/NEJMcp053024] [PMID]
[16] Martin JN Jr, Bailey AP, Rehberg JF, Owens MT, Keiser SD, May WL. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006. American Journal of Obstetrics and Gynecology. 2008; 199(2):98-104. [DOI:10.1016/j.ajog.2008.03.011] [PMID]
[17] Lattuada A, Rossi E, Calzarossa C, Candolfi R, Mannucci PM. Mild to moderate reduction of a Willebrand factor cleaving protease (ADAMS13) in pregnant women with HELLP microangiopathic syn- drome. Haematologica. 2003; 88(9):1029-34. [PMID]
[18] Molvarec A, Rigó J Jr, Bõze T, Derzsy Z, Cervenak L, Makó V, et al. Increased plasma von Willebrand factor cleaving protease (AD- AMS13) activity in preeclampsia. Thrombosis and Haemostasis. 2009; 101(2):305-11. [DOI:10.1160/TH08-05-0330] [PMID]
[19] Sayani FA, Abrams CS. How I treat refractory thrombotic throm- bocytopenic purpura. Blood. 2015; 125(25):3860-7. [DOI:10.1182/ blood-2014-11-551580] [PMID] [PMCID]
[20] Scully M, McDonald V, Cavenagh J, Hunt BJ, Longair I, Cohen H, et al. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombo- cytopenic purpura. Blood. 2011; 118(7):1746-53. [DOI:10.1182/ blood-2011-03-341131] [PMID]
[21] Chaturvedi S, Abbas H, McCrae KR. Increased morbidity during long-term follow-up of survivors of thrombotic thrombocytopenic purpura. American Journal of Hematology. 2015; 90(10):E208. [DOI:10.1002/ajh.24138] [PMID] [PMCID]
| Files | ||
| Issue | Vol 6 No S1 (2021): Supplementary | |
| Section | Case Report(s) | |
| Keywords | ||
| Thrombotic Thrombocytopenic Purpura Pregnancy Preeclampsia Plasma exchange sepsis | ||
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How to Cite
1.
Marsusi V, Jamal AS, Saleh M, Nouri B. Maternal Death Due to Refractory Thrombotic Thrombocytopenic Purpura (TTP) Associated With Superimposed Preeclampsia, Sepsis, and Intracranial Hemorrhage: A Case Report. CRCP. 2022;6(S1):292-297.
