ACUTE SPINAL CORD INFARCTION IN THE SETTING OF HEREDITARY THROMBOTIC THROMBOCYTOPENIC PURPURA

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP) is an acquired or inherited condition characterized by platelet aggregation and thrombosis due to accumulation of von Willebrand factor multimers caused decreased ADAMTS13 protease activity. It usually presents with fever, microangiopathic hemolytic anemia, thrombocytopenia, neurologic findings, renal dysfunction. Acquired TTP inhibitory autoantibody against treated glucocorticoids, rituximab, plasmapheresis, caplacizumab [1]. Hereditary autosomal recessive mutation prophylactic therapeutic plasma infusion [2]. Despite its prothrombotic nature, active bleeding at presentation uncommon complication described in literature. [3] We present unusual case hereditary that acute spinal cord infarction from into the canal. CASE PRESENTATION: A 33-year-old female past medical history systemic lupus erythematosus presented malaise, fevers, dyspnea, vomiting, diarrhea. She was altered febrile upon arrival, but then developed vagina, rectum, bladder. empirically diagnosed based on encephalopathy, lab work (hemolysis, kidney injury, thrombocytopenia). Blood product transfusions plasmapheresis were initiated. activity negative inhibitor testing, so infusions started lieu plasmapheresis. During her hospitalization she exhibited progressive diffuse lower extremity weakness, sensory deficits, absent deep tendon reflexes. MRI brain showed C5 conus medullaris, as well extra-arachnoid fluid collection/hematoma throughout dorsal canal C3 T12/L1. This a subdural hematoma causing severe stenosis proximal mid-thoracic not surgical candidate, aggressive physical therapy. DISCUSSION: challenging typically leads rather than bleeding, development manifestation for TTP. normal coagulation studies studies, patient's clinical confirmed even setting significant bleeding. With dysfunction, differential includes transverse myelitis, epidural abscess, infarction, syringomyelia. spine diagnostic. management must include transfusion it can be life saving.[3] CONCLUSIONS: differs whether acquired. Although entity, clinicians keep critically ill patient. REFERENCE #1: Scully M, Hunt BJ, Benjamin S, et al. Guidelines diagnosis thrombotic other microangiopathies. Br J Haematol 2012; 158:323. #2: Hamroun A, Prouteau C, Provôt F. Thrombocytopenic Purpura. N Engl Med 2020; 382:392. #3: Swisher KK, Terrell DR, Vesely SK, Clinical outcomes after patients purpura. Transfusion 2009; 49:873. DISCLOSURES: No relevant relationships Mohamed Hajmurad, source=Web Response Jonathan Hendrie, Hamza Khan, Daniel Matthews,