26 ‘hot seat’ cases in new transfusion medicine text

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• List the differential diagnoses of thrombotic microangiopathy (TMA).
• List the key features of thrombotic thrombocytopenic purpura (TTP), typical hemolytic uremic syndrome (HUS), and atypical HUS, versus secondary TMAs.
• Describe the management options in TTP and HUS.

References

1. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654-666.
2. George JN. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood. 2010;116(20):4060-4069.
3. Park YA, Hay SN, King KE, et al. Is it quinine TTP/HUS or quinine TMA? ADAMTS13 levels and implications for therapy. J Clin Apheresis. 2009;24(3):115-119.
4. Bennett CL, Connors JM, Carwile JM, et al. Thrombotic thrombocytopenic purpura associated with clopidogrel. N Engl J Med. 2000;342(24):1773-1777.
5. Zakarija A, Bandarenko N, Pandey DK, et al. Clopidogrel-associated TTP: an update of pharmacovigilance efforts conducted by independent researchers, pharmaceutical suppliers, and the Food and Drug Administration. Stroke. 2004;35(2):533-537.
6. Rock G, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 1991;325(6):393-397.
7. Garderet L, Maury E, Lagrange M, Najman A, Offenstadt G, Guidet B. Schizocytosis in pernicious anemia mimicking thrombotic thrombocytopenic purpura. Am J Med. 2003;114(5):423-425.
8. 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):1746-1753.
9. Goel R, Ness PM, Takemoto C, Krishnamurti L, King KE, Tobian AAR. Platelet transfusions in platelet consumptive disorders are associated with arterial thrombosis and in-hospital mortality. Blood. 2015;125(9):1470-1476.
10. Kremer Hovinga JA, Vesely SK, Terrell DR, Lammle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010;115(8):1500-1511.
11. Cataland SR, Holers VM, Geyer S, Yang S, Wu HM. Biomarkers of terminal complement activation confirm the diagnosis of aHUS and differentiate aHUS from TTP. Blood. 2014;123(24):3733-3738.
12. Chandler WL, Jelacic S, Boster DR, et al. Prothrombotic coagulation abnormalities preceding the hemolytic-uremic syndrome. N Engl J Med. 2002;346(1):23-32.
13. Wong CS, Jelacic S, Habeeb RL, Watkins SL, Tarr PI. The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med. 2000;342(26):1930-1936.
14. Dundas S, Murphy J, Soutar RL, Jones GA, Hutchinson SJ, Todd WTA. Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157:H7 outbreak. Lancet. 1999;354(9187):1327-1330.
15. Kielstein JT, Beutel G, Fleig S, et al. Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry. Nephrol Dial Transplant. 2012;27(10):3807-3815.

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