Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by thrombocytopenia, microan giopathic hemolytic anemia, renal dysfunction, fever, and neurologic symptoms. A specific von Willebrand factor-cleaving protease has recently been isolated from normal plasma. Patients with nonfamilial thrombotic thrombocytopenic purpura may have an acquired deficiency of this protease that is caused by an autoimmune mechanism.
We report a patient with thrombotic thrombocytopenic purpura following enterohemorrhagic
Escherichia coli O157 colitis with elevated platelet-associated IgG and antinuclear antibody.
A 50-year-old woman was admitted because of abdominal pain and bloody diarrhea. The following day, stool cultures obtained on admission were positive for
Escherichia coli O157. Hemorrhagic colitis was diagnosed, and treatment with antibiotics was initiated. On the fifth day of hospitalization, hemoglobin was 11.0g/d
l, platelet count was 26×10
3/μ
l and serum creatinine was 1.0mg/d
l with proteinuria and hematuria. A peripheral blood film showed red cell fragmentation. That evening her mental status deteriorated. Thrombotic thrombocytopenic purpura was diagnosed, and plasma exchange with fresh frozen plasma as replacement therapy was conducted. She received a total of six plasma exchanges.
The serum platelet-associated IgG level peaked at 249.0ng/10
7 cells on the 6th day of hospitalization and returned to normal by day 27th day. Lupus erythematosus test was also positive on the 6th day of hospitalization, but later returned to normal.
Based on these findings, thrombotic thrombocytopenic purpura in patients who have been diagnosed with enterohemorrhagic
Escherichia coli O157 may be caused by an autoimmune mechanism. We conclude that platelet-associated IgG and other antibodies may be also useful in the differential diagnosis of patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
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