Successful Treatment of Refractory Thrombotic Thrombocytopenic Purpura with Cyclosporine and Corticosteroids in a Patient with Systemic Lupus Erythematosus and Antibodies to ADAMTS13
Year: 2007
Type: article
Study design: Case Report
Abstract: A 46-year-old woman with systemic lupus erythematosus was hospitalized for purpura, hematochezia and hematuria. One week after admission, she developed grand mal seizures and coma and was diagnosed with thrombotic thrombocytopenic purpura (TTP) when fragmented red cells were found on the peripheral blood smear. Laboratory findings showed severe ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats) deficiency and anti-ADAMTS13 antibodies, which in recent reports have indicated a poor prognosis. She was refractory to methylprednisolone pulse therapy and plasma exchange, but administration of cyclosporine induced remission without adverse effects. We propose that cyclosporine may be an effective treatment for cases of refractory TTP.
Source: Internal Medicine
Authors: Terukazu Enami, Takeshi Suzuki, Satoshi Ito, Ai Yoshimi, Makoto Sugihara, Mizuko Mamura, Taichi Hayashi, Daisuke Goto, Isao Matsumoto, Akito Tsutsumi, Takayuki Sumida
Institution: University of Tsukuba
Language: English
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: bronze
OpenAlex ID: W1998728671