title: ADAMTS13 deficiency and thrombotic thrombocytopenic purpura associated with trimethoprim-sulfamethoxazole.
abstract: Thrombotic thrombocytopenic purpura (TTP) is a hematological disease characterized by microangiopathic hemolytic anemia and thrombocytopenia. Although the link between ADAMTS13 deficiency and idiopathic TTP has been well-established, the role of trimethoprim-sulfamethoxazole (TMP-SMX) in the pathogenesis of TTP is not yet well elucidated. To the best of our knowledge, there have been only two previous reports linking this medication with the development of TTP. We present the case of a healthy woman, age 26 years, who developed TTP during TMP-SMX therapy for urinary tract infection. She was found to have ADAMTS13 deficiency with anti-ADAMTS13 antibodies. Her condition responded to discontinuation of the TMP-SMX, plasmapheresis, and rituximab therapy. We speculate that the acquired ADAMTS13 deficiency might have been triggered by the TMP-SMX therapy.
authors.affiliation: Department of Internal Medicine, Marshfield Clinic, 1000 North Oak Avenue, Marshfield, WI 54449, USA. bapani.sowjanya@marshfieldclinic.org
authors.forename: Sowjanya
authors.initials: S
authors.lastname: Bapani
authors.forename: Narendranath
authors.initials: N
authors.lastname: Epperla
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Kasirye
authors.forename: Richard
authors.initials: R
authors.lastname: Mercier
authors.forename: Romel
authors.initials: R
authors.lastname: Garcia-Montilla
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000328:Adult; D000892:Anti-Infective Agents, Urinary / Q000009:adverse effects* / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D005260:Female; D006801:Humans; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000139:chemically induced* / Q000175:diagnosis*; D000069283:Rituximab; D016896:Treatment Outcome; D015662:Trimethoprim, Sulfamethoxazole Drug Combination / Q000009:adverse effects* / Q000627:therapeutic use; D014552:Urinary Tract Infections / Q000188:drug therapy; D028761:Withholding Treatment
chemical_list: D000892:Anti-Infective Agents, Urinary; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D015662:Trimethoprim, Sulfamethoxazole Drug Combination; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human