title: [A case of thrombotic thrombocytopenic purpura with systemic lupus erythematosus].
abstract: We described a case of thrombotic thrombocytopenic purpura (TTP) with systemic lupus erythematosus (SLE). A-60-year old woman was admitted to our hospital because of fever, disconsciousness, and general fatigue. 32 years ago, she was diagnosed as SLE with Raynaud's phenomenon, rash, photosensitivity, arthritis, lymphocytopenia, and ANA. Her SLE was well controlled with 10 mg predonisolone as a maintance dose until several weeks ago. On admission, severe thrombocytopenia (0.7x10(4)/microl) and other laboratory data revealed microangiopathic hemolytic anemia and renal dysfunction, Immediately after diagnosed as TTP, plasma exchange and corticosteroid therapy started. In spite of the treatment, disconsciousness progressed and systemic convulsion occurred and died 4 days after admission. Autopsied examination revealed diffuse microvascular hyalinized thrombi in heart, kidney, liver, spleen, and pancreas. Some microvascular thrombi were detected in lymph nodes, bone marrow, intestine. Pathological diagnosis of TTP was made on microvascular hyalinized platelet thrombi in organs. Von Willebrand factor-cleaving protease (VWF-CP) activity in plasma on set is less than 0.5 percent of normal and inhibitor for VWF-CP was detected. We here report a valuable case for analysis of pathogenesis in SLE-TTP.
authors.affiliation: Department of Pathology, Sapporo General City Hospital.
authors.forename: Yayoi
authors.initials: Y
authors.lastname: Ogawa
authors.forename: Masaya
authors.initials: M
authors.lastname: Mukai
authors.forename: Hideki
authors.initials: H
authors.lastname: Gotoh
authors.forename: Satoshi
authors.initials: S
authors.lastname: Tanaka
authors.forename: Akio
authors.initials: A
authors.lastname: Takada
authors.forename: Toshinao
authors.initials: T
authors.lastname: Takenouchi
mesh_terms: D051722:ADAM Proteins / Q000378:metabolism; D000071120:ADAMTS13 Protein; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology* / Q000473:pathology*
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human