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Published in: Journal of Medical Case Reports 1/2018

Open Access 01-12-2018 | Case report

Post-Partum Thrombotic Thrombocytopenic Purpura (TTP) in a Patient with known Idiopathic (Immune) Thrombocytopenic Purpura: a case report and review of the literature

Authors: Naser Al-Husban, Oqba Al-Kuran

Published in: Journal of Medical Case Reports | Issue 1/2018

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Abstract

Background

Incidences of immune thrombocytopenic purpura occur in 1 in every 1000–10,000 pregnancies accounting for 3% of all thrombocytopenic pregnancies. A pre-existing immune thrombocytopenic purpura is known to be a risk factor for developing thrombocytopenia during pregnancy. We present here the treatment regime and management of a patient with known immune thrombocytopenic purpura who developed postpartum thrombotic thrombocytopenia with atypical response to traditional therapy.
Pregnant women are more vulnerable to immune thrombocytopenic purpura or thrombotic thrombocytopenia. Pregnancy or postpartum thrombotic thrombocytopenia accounts for 10–25% of all thrombotic thrombocytopenia.

Case presentation

This case report deals with the treatment regime and management of a patient with known immune thrombocytopenic purpura who developed postpartum thrombotic thrombocytopenia. A 30-year-old Middle Eastern woman, with a prior diagnosis of chronic immune thrombocytopenic purpura had remained off-the-treatment for many years. After primary unexplained infertility for 8 years, for which she underwent six failed trials of in vitro fertilization, she delivered a healthy baby through caesarean section. Two days post-surgery, she had persistent thrombocytopenia, ecchymoses, bruises, and hemolysis. Her blood film revealed leukoerythroblastic anemia. Her blood tests also revealed a very low level of haptoglobin, and low level of ADAMTS13. A diagnosis of thrombotic thrombocytopenia was suspected. Plasma exchange therapy was started with partial response. We showed that rituximab in conjunction with mycophenolate mofetil following plasma exchange therapy was effective in controlling the low platelet count in our patient.

Conclusions

Rituximab in conjunction with mycophenolate mofetil following plasma exchange therapy was effective in controlling the low platelet count in our patient. Only two doses of rituximab were sufficient to normalize our patient. We present here a case of safe and effective use of rituximab in pregnancy-induced thrombotic thrombocytopenia.
Literature
1.
go back to reference 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(1):5–10.CrossRefPubMed 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(1):5–10.CrossRefPubMed
2.
go back to reference Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168–86.CrossRefPubMed Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168–86.CrossRefPubMed
3.
go back to reference Blombery P, Scully M. Management of thrombotic thrombocytopenic purpura: current perspectives. J Blood Med. 2014;5:15–23.PubMedPubMedCentral Blombery P, Scully M. Management of thrombotic thrombocytopenic purpura: current perspectives. J Blood Med. 2014;5:15–23.PubMedPubMedCentral
4.
go back to reference George JN, El-Harake MA, Raskob GE. Chronic idiopathic thrombocytopenic purpura. N Engl J Med. 1994;331:1207–11.CrossRefPubMed George JN, El-Harake MA, Raskob GE. Chronic idiopathic thrombocytopenic purpura. N Engl J Med. 1994;331:1207–11.CrossRefPubMed
5.
go back to reference Scaradavou A, Woo B, Woloski BM, et al. Intravenous anti-D treatment of immune thrombocytopenic purpura: experience in 272 patients. Blood. 1997;89:2689–700.PubMed Scaradavou A, Woo B, Woloski BM, et al. Intravenous anti-D treatment of immune thrombocytopenic purpura: experience in 272 patients. Blood. 1997;89:2689–700.PubMed
6.
go back to reference George JN. The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: evaluation, management, and long-term outcomes experience of the Oklahoma TTP-HUS Registry, 1989–2007. Kidney Int Suppl. 2009;112(S):52–4.CrossRef George JN. The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: evaluation, management, and long-term outcomes experience of the Oklahoma TTP-HUS Registry, 1989–2007. Kidney Int Suppl. 2009;112(S):52–4.CrossRef
7.
go back to reference George JN, Terrell DR, Swisher KK, Vesely SK. Lessons learned from the Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome registry. J Clin Apher. 2008;23(4):129–37.CrossRefPubMed George JN, Terrell DR, Swisher KK, Vesely SK. Lessons learned from the Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome registry. J Clin Apher. 2008;23(4):129–37.CrossRefPubMed
8.
go back to reference George JN. The association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Curr Opin Hematol. 2003;10:339–44.CrossRefPubMed George JN. The association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Curr Opin Hematol. 2003;10:339–44.CrossRefPubMed
9.
go back to reference 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(5):662–8.PubMed 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(5):662–8.PubMed
10.
go back to reference Stella CL, Dacus J, Guzman E, Dhillon P, Coppage K, et al. The diagnosis dilemma of thrombotic thrombocytopenic purpura/haemolytic uremic syndrome in the obstetric triage and emergency department: lessons from 4 tertiary hospitals. Am J Obstet Gynecol. 2009;200:381.e1–6.CrossRef Stella CL, Dacus J, Guzman E, Dhillon P, Coppage K, et al. The diagnosis dilemma of thrombotic thrombocytopenic purpura/haemolytic uremic syndrome in the obstetric triage and emergency department: lessons from 4 tertiary hospitals. Am J Obstet Gynecol. 2009;200:381.e1–6.CrossRef
11.
go back to reference McCrae KR. Thrombocytopenia in pregnancy. Educ Program Blood Haematology. 2010;2010:397–402. McCrae KR. Thrombocytopenia in pregnancy. Educ Program Blood Haematology. 2010;2010:397–402.
12.
13.
go back to reference Lesesve JF, Salignac S, Lecompte T. Laboratory measurement of schistocytes. Int J Lab Hematol. 2007;29(2):149–51.CrossRefPubMed Lesesve JF, Salignac S, Lecompte T. Laboratory measurement of schistocytes. Int J Lab Hematol. 2007;29(2):149–51.CrossRefPubMed
14.
go back to reference Mashrafi A, Patel AR. Evaluation of normal reference range of schistocytes and burr Cells in Healthy Adults. Blood. 2015;126:4540. Mashrafi A, Patel AR. Evaluation of normal reference range of schistocytes and burr Cells in Healthy Adults. Blood. 2015;126:4540.
15.
go back to reference Curiel RV, Bhagati R, Basavaraju L, et al. VonWillebrand factor, red cell fragmentation, and disease activity in systemic lupus erythematosus. HSS J. 2008;4(2):170–4.CrossRefPubMedPubMedCentral Curiel RV, Bhagati R, Basavaraju L, et al. VonWillebrand factor, red cell fragmentation, and disease activity in systemic lupus erythematosus. HSS J. 2008;4(2):170–4.CrossRefPubMedPubMedCentral
16.
go back to reference Martin JN, Bailey AP, Rehberg JF, et al. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006. Am J Obstet Gynecol. 2008;199:98–104.CrossRefPubMed Martin JN, Bailey AP, Rehberg JF, et al. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006. Am J Obstet Gynecol. 2008;199:98–104.CrossRefPubMed
17.
go back to reference Macken E, Lewi L, Dierickx D. Thrombotic thrombocytopenic purpura in pregnancy: a case report. Belg J Hematol. 2014;5(3):106–9. Macken E, Lewi L, Dierickx D. Thrombotic thrombocytopenic purpura in pregnancy: a case report. Belg J Hematol. 2014;5(3):106–9.
18.
go back to reference Crowther MA, George JN. Thrombotic thrombocytopenic purpura: 2008 update. Cleve Clin J Med. 2008;75(5):369–75.CrossRefPubMed Crowther MA, George JN. Thrombotic thrombocytopenic purpura: 2008 update. Cleve Clin J Med. 2008;75(5):369–75.CrossRefPubMed
19.
go back to reference Pereira A, Mazzara R, Monteagudo J, et al. Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: a multivariate analysis of factors predicting the response to plasma exchange. Ann Hematol. 1995;70(6):319–23.CrossRefPubMed Pereira A, Mazzara R, Monteagudo J, et al. Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: a multivariate analysis of factors predicting the response to plasma exchange. Ann Hematol. 1995;70(6):319–23.CrossRefPubMed
20.
go back to reference Tulpule SA, Francis YA, Radia D, Harrison CN, Hunt BJ. Safety and Efficacy of Mycophenolate Mofetil in Relapsing Acquired Thrombotic Thrombocytopenic Purpura: Could It Prevent Further Relapse? Blood. 2006;108:3993. Tulpule SA, Francis YA, Radia D, Harrison CN, Hunt BJ. Safety and Efficacy of Mycophenolate Mofetil in Relapsing Acquired Thrombotic Thrombocytopenic Purpura: Could It Prevent Further Relapse? Blood. 2006;108:3993.
21.
go back to reference Zimmer-Molsberger B, Knauf W, Thiel E. Mycophenolate mofetil for severe autoimmune haemolytic anaemia. Lancet. 1997;359:1003–4.CrossRef Zimmer-Molsberger B, Knauf W, Thiel E. Mycophenolate mofetil for severe autoimmune haemolytic anaemia. Lancet. 1997;359:1003–4.CrossRef
22.
go back to reference Howard J, Hoffbrand AV, Prentice HG, Mehta A. Mycophenolate mofetil for the treatment of refractory auto-immune haemolytic anaemia and auto-immune thrombocytopenia purpura. Br J Haematol. 2002;117:712–5.CrossRefPubMed Howard J, Hoffbrand AV, Prentice HG, Mehta A. Mycophenolate mofetil for the treatment of refractory auto-immune haemolytic anaemia and auto-immune thrombocytopenia purpura. Br J Haematol. 2002;117:712–5.CrossRefPubMed
23.
go back to reference Scully M, McDonald V, Cavenagh J, et al. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 2011;118(7):1746–53.CrossRefPubMed Scully M, McDonald V, Cavenagh J, et al. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 2011;118(7):1746–53.CrossRefPubMed
24.
go back to reference Stasi R, Pagano A, Stipa E, Amadori S. Rituximab chimaeric anti-CD20 monoclonal antibody treatment for adults with chronic idiopathic thrombocytopenic purpura. Blood. 2001;98:952–7.CrossRefPubMed Stasi R, Pagano A, Stipa E, Amadori S. Rituximab chimaeric anti-CD20 monoclonal antibody treatment for adults with chronic idiopathic thrombocytopenic purpura. Blood. 2001;98:952–7.CrossRefPubMed
25.
go back to reference Chakravarty EF, Murray ER, Kelman A, Framer P. Pregnancy outcomes after maternal exposure to rituximab. Blood. 2011;117:1499–506.CrossRefPubMed Chakravarty EF, Murray ER, Kelman A, Framer P. Pregnancy outcomes after maternal exposure to rituximab. Blood. 2011;117:1499–506.CrossRefPubMed
26.
go back to reference Scully M, Starke R, Lee R, et al. Successful management of pregnancy in women with a history of thrombotic thrombocytopaenic purpura. Blood Coagul Fibrinolysis. 2006;17:459–63.CrossRefPubMed Scully M, Starke R, Lee R, et al. Successful management of pregnancy in women with a history of thrombotic thrombocytopaenic purpura. Blood Coagul Fibrinolysis. 2006;17:459–63.CrossRefPubMed
27.
go back to reference McDonald V, Manns K, Mackie IJ, Machin SJ, Scully MA. Rituximab pharmacokinetics during the management of acute idiopathic thrombotic thrombocytopenic purpura. J Thromb Haemost. 2010;8(6):1201–8.CrossRefPubMed McDonald V, Manns K, Mackie IJ, Machin SJ, Scully MA. Rituximab pharmacokinetics during the management of acute idiopathic thrombotic thrombocytopenic purpura. J Thromb Haemost. 2010;8(6):1201–8.CrossRefPubMed
Metadata
Title
Post-Partum Thrombotic Thrombocytopenic Purpura (TTP) in a Patient with known Idiopathic (Immune) Thrombocytopenic Purpura: a case report and review of the literature
Authors
Naser Al-Husban
Oqba Al-Kuran
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2018
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-018-1692-1

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