title: A case series of atypical presentations of thrombotic thrombocytopenic purpura.
abstract: Thrombotic thrombocytopenic purpura (TTP) is a heterogeneous disease primarily characterized by thrombocytopenia and microangiopathic hemolytic anemia. Therapeutic plasma exchange has dramatically improved mortality, allowing for emergence of refractory, relapsing, and atypical presentations. In this article, we describe four cases of TTP presenting with minimal schistocytes, mild elevation of lactate dehydrogenase, and symptoms suggestive of macrovascular arterial involvement. With increasing reports of less common presentations of TTP, clinicians should consider this diagnosis in cases of unexplained arterial thrombosis, thrombocytopenia, or hemolytic anemia. Testing for a disintegrin and metalloprotease with thrombospondin Type 1 motif, Member 13 ADAMTS13 activity was extremely useful to help confirm the diagnosis in our series of patients.
authors.affiliation: Division of Hematology/Oncology, Department of Medicine, University of Florida, Gainesville, Florida 32610, USA.
authors.forename: Iman
authors.initials: I
authors.lastname: Imanirad
authors.forename: Anita
authors.initials: A
authors.lastname: Rajasekhar
authors.forename: Marc
authors.initials: M
authors.lastname: Zumberg
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008875:Middle Aged; D010951:Plasma Exchange; D010976:Platelet Count; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000097:blood / Q000175:diagnosis / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D012189:Retrospective Studies; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human