We report a female patient who presented with generalized weakness, episodes of altered mental status and slurred speech, and a history of systemic lupus erythematosus. Initial investigations showed profound thrombocytopenia and schistocytosis on peripheral blood smear. PLASMIC score was promptly calculated, and plasma exchange with steroids was initiated based on the initial high PLASMIC score. Bone marrow examination showed hypocellular marrow without any other obvious abnormalities. The patient's platelet counts initially improved but had a quick decline, on which, rituximab and subsequently caplacizumab were introduced. The patient was discharged after stabilization with plasma exchange (PLEX) therapy as needed on an outpatient basis.
Keywords: caplacizumab; plasmic score; refractory thrombotic thrombocytopenic purpura; rituximab; systemic lupus erythematosus.
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