Sequential Immune Thrombocytopenia (ITP) and Thrombotic Thrombocytopenic Purpura (TTP) in an Elderly Male Patient with Primary Sjogren’s Syndrome: When in Doubt, Use the PLASMIC Score

Abstract

Introduction. Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy due to an acquired autoantibody to ADAMTS13 that requires a boutique treatment, urgent plasma exchange. Thus, TTP is often termed a “cannot miss” diagnosis. Case. We report a patient with TTP who had a history of immune thrombocytopenia (ITP), had atypical demographics for TTP, and had also met criteria for primary Sjogren’s syndrome. This exceedingly rare combination presented a temptation to dismiss TTP as a diagnosis. Discussion. Our case further demonstrates the practical utility of using the PLASMIC score as a tool that can help identify patients with TTP even when the patient has statistically rare characteristics.