Aurora St. Luke's Medical Center

Aurora Sinai Medical Center

Presentation Notes

Poster presented at 2018 APC Wisconsin Chapter Annual Scientific Meeting; September 7, 2018; Wisconsin Dells, WI.


Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic micro-angiopathy that classically presents with a pentad of microangiopathic hemolytic anemia, thrombocytopenic purpura, neurological dysfunction, fever and renal disease. Onset is usually acute to sub-acute. This pentad is only present in about 40% of cases which makes diagnosis of this rare condition challenging especially in cases with pre-existing conditions. Making the diagnosis of TTP is crucial as this condition has a 90% mortality rate if left untreated. Case Description: 36 y/o F with a past medical history of bilateral pulmonary embolism, systemic lupus erythematosus, and bipolar disorder presented to ED with concerns of hypotension; however, that was quickly ruled out. Her lab work was consistent with acute kidney injury (creatinine of 2.5mg/dL), anemia (HGB of 7.0mg/dL), and thrombocytopenia (15k/ mcL). The patient had no active complaints at the time of admission without fever and no overt signs of bleeding on physical exam. Neurological exam was normal. Patient appeared to be manic as she had pressured speech, difficulty concentrating and was hyperactive. Upon further investigation, she was found to have worsening of proteinuria with urine protein creatinine ratio of 14,639mPR/gCR. Other labs, including peripheral smear, LDH, Haptoglobin, Coombs test, d-dimer, fibrinogen, direct and indirect bilirubin, ADAMTS 13 activity and inhibitor were collected. Initial labs were consistent with hemolytic anemia. She was initially started on treatment for immune thrombocytopenic purpura ( ITP ) due to preexisting autoimmune disease, however that diagnosis was ruled out given negative Coombs test. About 4 days later, the ADAMTS 13 activity came back at <5% . She was subsequently started on plasma exchange therapy and clinically improved. Conclusion: TTP is a rare diagnosis however it should remain on the differential diagnosis of a patient with anemia and thrombocytopenia until completely ruled out. Treatment should be started prior to known ADAMTS13 activity results as delaying treatment could be life-threatening.

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