Acquired Thrombotic Thrombocytopenic Purpura and Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab

• DOI: 10.1080/08998280.2017.11929576
• Publication Date: 2017-12-11T19:03:29Z
• Authors: Appalanaidu Sasapu; Michele Cottler-Fox; Pooja Motwani
• Keywords: Eculizumab; Schistocyte; Thrombotic microangiopathy; Purpura (gastropod)

Acquired idiopathic thrombotic thrombocytopenic purpura is a life-threatening disease with mortality of up to 90%, if not promptly recognized and treated. We report 64-year-old woman this condition who presented left-sided weakness seizure-like activity preceded by headache easy bruising. She did achieve optimal response plasma exchange, corticosteroids, rituximab, vincristine. initiated treatment eculizumab, following which she had durable remission that continued for 30 months after discontinuation the drug. later found our patient has homozygous deletion in two closely related genes, complement factor H-related 1 3.