Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening autoimmune or hereditary thrombotic microangiopathy (TMA) that may be difficult to recognize given the wide spectrum of presenting symptoms. The clinical diagnosis of TTP is based on thrombocytopenia, microangiopathic hemolytic anemia and is confirmed by a disintegrin-like and metalloproteinase with thrombospondin type one motif, member 13 (ADAMTS13) <10%. However, the latter confirmation is not rapidly available, and treatment is typically initiated based on the degree of clinical suspicion. The PLASMIC score was recently developed to distinguish between TMA patients with and without severe ADAMTS13 deficiency and used as an adjunct in the diagnosis of TTP when the clinical picture is not clear. Here we present the case of a completely asymptomatic female with no past medical history diagnosed with TTP after evaluation for thrombocytopenia found on a routine wellness visit. A high PLASMIC score was crucial in the decision to initiate treatment given an unusual asymptomatic presentation.