Case Report

ST-Segment-Elevation Myocardial Infarction Unmasking Underlying Systemic Lupus Erythematosus or Representing Thrombotic Thrombocytopenic Purpura? Report of a Challenging Case

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder that frequently manifests itself with renal and neurological involvements. Cardiac involvement, however, has been rarely reported. In this report, we present a rare case of acquired TTP with acute myocardial infarction (AMI) as the initial manifestation. Although AMI was successfully managed by percutaneous coronary intervention, the patient developed hemolytic anemia, fever, marked thrombocytopenia, oliguria, and renal dysfunction, requiring treatment with plasma exchange and corticosteroids. TTP, albeit extremely rare, should be considered in cases with unexpected thrombocytopenia during acute-phase treatment for AMI as it can be highly lethal if not treated immediately.

1. Wiernek SL, Jiang B, Gustafson GM, Dai X. Cardiac implications of thrombotic thrombocytopenic purpura. World J Cardiol 2018;10:254-266.
2. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U, Solenthaler M, Lämmle B. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998;339:1578-1584.
3. Hosler GA, Cusumano AM, Hutchins GM. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases. Arch Pathol Lab Med 2003;127:834-839.
4. Ridolfi RL, Bell WR. Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature. Medicine (Baltimore) 1981;60:413-428.
5. Doll JA, Kelly JP. ST-segment elevation myocardial infarction treated with thrombolytic therapy in a patient with thrombotic thrombocytopenic purpura. J Thromb Thrombolysis 2014;38:124-126.
6. Blum D, Blake G. Lupus-associated thrombotic thrombocytopenic purpura-like microangiopathy. World J Nephrol 2015;4:528-531.
7. Hawkins BM, Abu-Fadel M, Vesely SK, George JN. Clinical cardiac involvement in thrombotic thrombocytopenic purpura: a systematic review. Transfusion 2008;48:382-392.
8. Gandhi K, Aronow WS, Desai H, Amin H, Sharma M, Lai HM, Singh P. Cardiovascular manifestations in patients with thrombotic thrombocytopenic purpura: a single-center experience. Clin Cardiol 2010;33:213-216.
9. Gami AS, Hayman SR, Grande JP, Garovic VD. Incidence and prognosis of acute heart failure in the thrombotic microangiopathies. Am J Med 2005;118:544-547.
10. McCarthy LJ, Danielson CF, Skipworth EM, Peters SL, Miraglia CC, Antony AC. Myocardial infarction/injury is relatively common at presentation of acute thrombotic thrombocytopenic purpura: the Indiana University experience. Ther Apher 2002;6:2-4.
11. Patschan D, Witzke O, Dührsen U, Erbel R, Philipp T, Herget-Rosenthal S. Acute myocardial infarction in thrombotic microangiopathies--clinical characteristics, risk factors and outcome. Nephrol Dial Transplant 2006;21:1549-1554.
12. Pene F, Vigneau C, Auburtin M, Moreau D, Zahar JR, Coste J, Heshmati F, Mira JP. Outcome of severe adult thrombotic microangiopathies in the intensive care unit. Intensive Care Med 2005;31:71-78.
13. Tobbia P, Brodie BR, Witzenbichler B, Metzger C, Guagliumi G, Yu J, Kellett MA, Stuckey T, Fahy M, Mehran R, Stone GW. Adverse event rates following primary PCI for STEMI at US and non-US hospitals: three-year analysis from the HORIZONS-AMI trial. EuroIntervention 2013;8:1134-1142.
14. Zakarija A, Bandarenko N, Pandey DK, Auerbach A, Raisch DW, Kim B, Kwaan HC, McKoy JM, Schmitt BP, Davidson CJ, Yarnold PR, Gorelick PB, Bennett CL. Clopidogrel-associated TTP: an update of pharmacovigilance efforts conducted by independent researchers, pharmaceutical suppliers, and the Food and Drug Administration. Stroke 2004;35:533-537.
15. Bennett CL, Connors JM, Carwile JM, Moake JL, Bell WR, Tarantolo SR, McCarthy LJ, Sarode R, Hatfield AJ, Feldman MD, Davidson CJ, Tsai HM. Thrombotic thrombocytopenic purpura associated with clopidogrel. N Engl J Med 2000;342:1773-1777.
16. Fujimura Y, Matsumoto M. Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008. Intern Med 2010;49:7-15.
17. Mannucci PM, Vanoli M, Forza I, Canciani MT, Scorza R. Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis). Haematologica 2003;88:914-918.
18. Matsuyama T, Kuwana M, Matsumoto M, Isonishi A, Inokuma S, Fujimura Y. Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases. Thromb Haemost 2009;102:371-378.
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IssueVol 16 No 2 (2021): J Teh Univ Heart Ctr QRcode
SectionCase Report(s)
DOI https://doi.org/10.18502/jthc.v16i2.7391
Keywords
Purpura; thrombotic thrombocytopenic Lupus erythematosus; systemic Myocardial infarction ST elevation myocardial infarction

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How to Cite
1.
Ghodsi S, Jenab Y, Mohebi M, Kamranzadeh H, Mohammadi Z. ST-Segment-Elevation Myocardial Infarction Unmasking Underlying Systemic Lupus Erythematosus or Representing Thrombotic Thrombocytopenic Purpura? Report of a Challenging Case. J Tehran Heart Cent. 2021;16(2):84-88.