Intensive Care Medicine

, Volume 44, Issue 6, pp 893–896 | Cite as

Does this patient with thrombotic thrombocytopenic purpura have a cardiac involvement?

  • Lara Zafrani
  • Lene Russell
  • Elie AzoulayEmail author
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Clinical vignette

A 53-year-old man was admitted to the intensive care unit (ICU) for thrombotic microangiopathy (TMA). His past medical history included hypercholesterolemia and smoking. In addition to profound thrombocytopenia (7 × 109/l) and mechanical haemolytic anemia (positive schistocytes on the blood smear and negative direct antiglobulin test), he exhibited diffuse purpura on his lower extremities. There was neither chest pain nor clinical features of congestive heart failure, and the electrocardiogram (ECG) was normal. However, cardiac troponin I measurement was 0.45 µg/l. The diagnosis of thrombotic thrombocytopenic purpura (TTP) was immediately suspected, and plasma exchange (PEX) and steroids were initiated within 3 h of ICU admission. The diagnosis was confirmed by undetectable ADAMTS-13 activity on a sample drawn prior to the first PEX. On day 2, the patient presented with atrial fibrillation. An echocardiogram revealed global left ventricular hypokinesis with a...


  1. 1.
    Mannucci PM (2015) Understanding organ dysfunction in thrombotic thrombocytopenic purpura. Intensive Care Med 41:715–718. CrossRefPubMedGoogle Scholar
  2. 2.
    Moake JL (2002) Thrombotic microangiopathies. N Engl J Med 347:589–600. CrossRefPubMedGoogle Scholar
  3. 3.
    Joly BS, Coppo P, Veyradier A (2017) Thrombotic thrombocytopenic purpura. Blood 129:2836–2846. CrossRefPubMedGoogle Scholar
  4. 4.
    Peigne V, Perez P, Resche Rigon M et al (2012) Causes and risk factors of death in patients with thrombotic microangiopathies. Intensive Care Med 38:1810–1817. CrossRefPubMedGoogle Scholar
  5. 5.
    Brain MC (1978) An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries (Moschcowitz 1925). Thromb Haemost 40:9–10PubMedGoogle Scholar
  6. 6.
    Benhamou Y, Boelle P-Y, Baudin B et al (2015) Cardiac troponin-I on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura. Experience of the French Thrombotic Microangiopathies Reference Center. J Thromb Haemost JTH 13:293–302. CrossRefPubMedGoogle Scholar
  7. 7.
    Mariotte E, Blet A, Galicier L et al (2013) Unresponsive thrombotic thrombocytopenic purpura in critically ill adults. Intensive Care Med 39:1272–1281. CrossRefPubMedGoogle Scholar
  8. 8.
    Hosler GA, Cusumano AM, Hutchins GM (2003) Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases. Arch Pathol Lab Med 127:834–839.;2 PubMedCrossRefGoogle Scholar
  9. 9.
    Ridolfi RL, Hutchins GM, Bell WR (1979) The heart and cardiac conduction system in thrombotic thrombocytopenic purpura. A clinicopathologic study of 17 autopsied patients. Ann Intern Med 91:357–363CrossRefPubMedGoogle Scholar
  10. 10.
    Tasaki T, Yamada S, Nabeshima A et al (2015) An autopsy case of myocardial infarction due to idiopathic thrombotic thrombocytopenic purpura. Diagn Pathol 10:52. CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Balasubramaniyam N, Kolte D, Palaniswamy C et al (2013) Predictors of in-hospital mortality and acute myocardial infarction in thrombotic thrombocytopenic purpura. Am J Med 126(1016):e1–e7. CrossRefGoogle Scholar
  12. 12.
    Hawkins BM, Abu-Fadel M, Vesely SK, George JN (2008) Clinical cardiac involvement in thrombotic thrombocytopenic purpura: a systematic review. Transfusion (Paris) 48:382–392. CrossRefGoogle Scholar
  13. 13.
    Hughes AK, Stricklett PK, Padilla E, Kohan DE (1996) Effect of reactive oxygen species on endothelin-1 production by human mesangial cells. Kidney Int 49:181–189CrossRefPubMedGoogle Scholar
  14. 14.
    Morici N, Cantoni S, Panzeri F et al (2017) von Willebrand factor and its cleaving protease ADAMTS13 balance in coronary artery vessels: lessons learned from thrombotic thrombocytopenic purpura. A narrative review. Thromb Res 155:78–85. CrossRefPubMedGoogle Scholar
  15. 15.
    Georgiades F, Demosthenous S, Braimi M et al (2015) Takotsubo cardiomyopathy and thrombotic thrombocytopenic purpura preceding a lupus diagnosis: a case report. Lupus 24:1443–1447. CrossRefPubMedGoogle Scholar
  16. 16.
    Scully M, Hunt BJ, Benjamin S et al (2012) Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol 158:323–335. CrossRefPubMedGoogle Scholar
  17. 17.
    Peyvandi F, Scully M, Kremer Hovinga JA et al (2016) Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med 374:511–522. CrossRefPubMedGoogle Scholar
  18. 18.
    Gami AS, Hayman SR, Grande JP, Garovic VD (2005) Incidence and prognosis of acute heart failure in the thrombotic microangiopathies. Am J Med 118:544–547. CrossRefPubMedGoogle Scholar
  19. 19.
    Maino A, Siegerink B, Lotta LA, Crawley JT, le Cessie S, Leebeek FW, Lane DA, Lowe GD, Peyvandi F, Rosendaal FR (2015) Plasma ADAMTS-13 levels and the risk of myocardial infarction: an individual patient data meta-analysis. J Thromb Haemost 13(8):1396–1404CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature and ESICM 2018

Authors and Affiliations

  1. 1.AP-HP, Saint-Louis Hospital, Medical Intensive Care Unit, Paris (LZ, EA), ECSTRA Team, Biostatistics and Clinical Epidemiology, UMR 1153 INSERMParis Diderot, Sorbonne UniversityParisFrance
  2. 2.Department of Intensive Care 4131Copenhagen University Hospital, RigshospitaletCopenhagenDenmark
  3. 3.Department of AnaesthesiaZealand University HospitalRoskildeDenmark

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