title: Refractory thrombotic thrombocytopenic purpura associated with primary Sjogren syndrome treated with rituximab: a case report.
abstract: Thrombotic thrombocytopenic purpura (TTP) is an uncommon, serious disease that involves multiple organs and is rapidly fatal if left untreated. TTP is associated with multisystem symptoms, such as thrombocytopenia, microangiopathic hemolytic anemia, renal impairment, central nervous system involvement, and fever. TTP is idiopathic in about 37% of the cases and can be associated with autoimmune diseases in 13% of the cases. Autoimmune disease-associated TTP can be refractory to plasma exchange and requires immunosuppressive therapy. We report a case of a previously healthy 55-year-old African American female who presented with shortness of breath, hemolytic anemia, renal impairment, and thrombocytopenia. The diagnosis of TTP was made, and plasmapheresis was initiated. However, recurrence happened 48 hours after plasmapheresis was stopped. Autoimmune workup for refractory TTP revealed positive antinuclear antibodies, Anti-SSA, and Anti-SSB. Lip biopsy revealed findings consistent with Sjogren syndrome. Treatment with Rituximab was started, and significant clinical and laboratory response was achieved. The patient remained asymptomatic thereafter. A high clinical suspicion of autoimmune diseases is important as TTP tends to be refractory to plasma exchange in these cases, and immunosuppressive therapy is a key.
authors.affiliation: 1Department of Internal Medicine, University of Toledo Medical Center, Toledo, OH; and 2Department of Internal Medicine, St Vincent Mercy Medical Center, Toledo, OH.
authors.forename: Anis
authors.initials: A
authors.lastname: Toumeh
authors.forename: Navpreet
authors.initials: N
authors.lastname: Josh
authors.forename: Rawan
authors.initials: R
authors.lastname: Narwal
authors.forename: Ragheb
authors.initials: R
authors.lastname: Assaly
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008875:Middle Aged; D010956:Plasmapheresis / Q000379:methods; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy* / Q000209:etiology; D012008:Recurrence; D000069283:Rituximab; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab