Abstract

Introduction: Thrombotic Thrombocytopenic Purpura (TTP) is a fatal condition that can be induced by medications. NSAIDs are one of the medications known for this complication. Nimesulide is a banned NSAID in the USA and as per our knowledge one case of TTP associated with Nimisulide has been reported which resolved with a week of steroids and Plasma Exchange (PEX). Description: 46-year-old hispanic male with a history of migraine came to ER after a fall and right facial droop. The patient took Nimisulide 100 mg every 4 hours over the past three days for knee pain. He had headache, dizziness, blurry vision, confusion and petechiae on presentation. His stroke workup was negative. He was sedated for severe agitation and intubated for airway protection. His labs revealed severe thrombocytopenia, microangiopathic hemolytic anemia, elevated INR and D-Dimer, low fibrinogen, and acute kidney injury. The PLASMIC score was 7, TTP was suspected, and treatment was started with PEX and high dose steroids. He was treated empirically for meningitis and tick-borne illness, but his workup for infection, including a lumbar puncture was unremarkable and flowcytometry did not reveal hematologic malignancy. With clinical improvement, we switched steroids to the standard dose and extubated the patient. Initial ADAMTS13 activity was < 3%. After a transient response, the patient’s platelet count plummeted, and he developed worsening agitation. He had refractory disease, for which we started him on twice daily PEX, weekly Rituximab, and daily Caplacizumab. After that, he continued to show neurological and platelet count improvement. ADAMTS13 activity improved to 8% with inhibitor level 1.3 U/ml, and we monitored it biweekly. Over the next few weeks, we tapered PEX to once weekly, switched steroids to the standard dose, and Caplacizumab to once weekly. The current plan is to taper steroids once ADAMTS13 exceeds 30%. Discussion: This is a case of Nimesulide induced TTP which was refractory to the initial management with PEX and steroids. We report this case to raise awareness amongst physicians towards NSAID induced TTP. Further study is needed to determine if these patients should avoid NSAIDs for life. We also need additional data to comment on the clinical course of similar patients.

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