Mr. R did exhibit four of the five signs, but did not have any neurologic deficits. TTP is often seen in conjunction with hemolytic uremic syndrome (HUS). The incidence of suspected TTP-HUS is 11 cases/million/year. There is a greater propensity for the disease in women and blacks.



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In cases that are not idiopathic, etiology may be related to use of quinine, chemotherapeutic agents, immunosuppressive agents (e.g., cyclosporine,) and antiplatelet agents (e.g., clopidogrel and ticlopidine).2-4 Infectious causes are also noted to yield TTP-HUS. Shiga toxin released from E. coli can lead to HUS, especially in children.5 Thrombotic microangiopathy has been seen in HIV infection, and pneumococcal infection can also cause HUS in rare instances.6 TTP-HUS can be related to pregnancy or can be induced by oral contraceptives,7 and incidence has been linked to pancreatitis8 and to surgery.9

6. Summary


Left untreated, TTP-HUS can progress to renal failure, neurologic deterioration, cardiac ischemia and death. With the advent of plasma exchange, the mortality rate for this disorder has decreased from 90% to 20% in modern times. Mr. R received immediate plasmapheresis, which dramatically increased his platelet count. In patients with idiopathic TTP, as here, treatment with immunosuppressive agents such as prednisone is also recommended.1


After resolution of the acute TTP event, patients should be monitored with CBCs and LDH levels. In a study involving 63 subjects, the estimated 10-year relapse rate was 36%.10

Michele D. Tinge, MPAS, PA-C, is a physician assistant at Alton Internal Medicine in Alton, Ill.


References


1. George JN. “How I treat patients with thrombotic thrombocytopenic purpura.” Blood. 2010;116:4060-4069.


2. Kojouri K, Vesely SK, George JN. “Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: frequency, clinical features and long term outcomes.” Ann Intern Med. 2001;135:1037-1041.


3. Lesesne JB, Rothschild N, Erickson B et al. “Cancer-associated hemolytic uremic syndrome: analysis of 85 cases from a national registry.” J Clin Oncol. 1989;7:781-789.


4. Walfe JA, McCann RL, Sanfilippo F. “Cyclosporine-associated micro­angiopathy in renal transplantation: a severe but potentially reversible form of early graft injury.” Transplantation. 1986;41:541-544.


5. Boyce TG, Swerdlow DL, Griffin PM. “Escherichia coli 0157:H7 and the hemolytic uremic syndrome.” N Engl J Med. 1995;333:364-368.


6. Gervasoni C, Ridolfo AL, Vaccarezza M et al. “Thrombotic micro­angiopathy in patients with acquired immunodeficiency syndrome before and during the era of introduction of highly active antiretroviral therapy.” Clin Infect Dis. 2002;35:1534-1540.


7. Hauglustaine D, Van Damme B, Vanrenterghem Y, et al. “Recurrent hemolytic uremic syndrome during oral contraception.” Clin Nephrol. 1981;15:148-153.


8. Boyer A, Chadda K, Salah A, et al. “Thrombotic micro­angiopathy: an atypical cause of acute renal failure in patients with acute pancreatitis.” Intensive Care Med. 2004;30:1235-1239.


9. Chang JC, Shipstone A, Llenado-Lee MA. “Postoperative thrombotic thrombocytopenic purpura following cardiovascular surgeries.” Am J Hematol. 1996;53:11-19.


10. Ruggenenti P, Galbusera M, Cornejo RP et al. “Thrombotic thrombocytopenic purpura: evidence that infusion rather than removal of plasma induces remission of the disease.” Am J Kidney Dis. 1993;21:314-318.