Abstract Introduction Thrombotic thrombocytopenic purpura (TTP) is a microangiopathy characterized by severely reduced activity of ADAMTS13, a von Willebrand factor-cleaving protease. The incidence is approximately 3 per 1 million adults per year, making it a fairly rare diagnosis. While ADAMTS13 activity can confirm a diagnosis, testing results often take several days to finalize. Without treatment, TTP is almost always fatal. However, survival rate is greater than 90% with proper management, making the early stages of diagnosis and management crucial. Case Presentation Patient presented for evaluation for acute clumsiness, left-sided facial droop, and slurred speech. She also noted blood-tinged urine for 1 week. Initially, stroke was worked up, and MRI showed no acute infarction. Labs revealed an unremarkable complete metabolic panel, hemoglobin 8.7, platelet count of 19,000, and elevated total bilirubin at 3. Symptoms initially resolved, but recurred episodically while she was being treated. Due to negative stroke workup and suspected hematologic abnormality, the patient was given high dose IV steroids and blood products. The patient was then transferred for hematology evaluation. Further workup revealed hemolytic anemia with elevated indirect bilirubin, elevated LDH, decreased haptoglobin. Peripheral blood smear was obtained showing rare schistocytes with no other abnormalities. DAT testing was negative. Plasmic score was calculated at 6. Expressive aphasia and right upper extremity weakness occurred episodically. That same day, the patient had a vascular catheter placed and received plasma exchange. Platelet count trended up throughout her hospitalization and was 203,000 at the time of discharge. She received 3 days of plasma exchange and her first dose of rituximab as well as oral steroids prior to discharge. ADAMTS13 results, obtained on day of admission, resulted on the day of her discharge and showed severely decreased activity. Discussion TTP is a rare microangiopathy characterized by decreased activity of ADAMTS13. Immune TTP, the most common cause, can be linked to lupus, scleroderma, and other autoimmune conditions. TTP can also be linked to pregnancy and sometimes medications. The Plasmic score mentioned above can help risk stratify the likelihood of patients having TTP. Prompt recognition and early initiation of plasma exchange prior to the results of confirmatory testing can be lifesaving. Conclusion This case emphasizes the importance of maintaining a high index of suspicion for TTP in patients with unexplained neurological abnormalities and thrombocytopenia. Early involvement of multidisciplinary teams is essential to achieve an optimal outcome for the patient. This abstract is funded by: None
G T Tobin (Fri,) studied this question.