[Successful treatment with rituximab in a patient with refractory thrombotic thrombocytopenic purpura refractory to plasma exchange].
We report a patient with refractory idiopathic thrombotic thrombocytopenic purpura (TTP) who was successfully treated with rituximab. A 50-year-old woman was referred to our hospital with progressive psychoneurotic symptoms, hemolytic anemia and thrombocytopenia. The diagnosis of TTP was confirmed by the absence of ADAMTS13 activity with the presence of circulating ADAMTS13 inhibitor. High-dose steroid therapy and plasma exchange were performed. Despite 21 sessions of plasma exchange, however, there was no remarkable improvement. We then administered rituximab. Fifteen days after the first infusion of rituximab, she achieved complete remission and ADAMTS13 activity increased up to 14%. The patient has remained in remission for more than 9 months.
Koshino
Mayuko
M
Division of Hematology, Institute of Clinical Medicine, University of Tsukuba.
Kudou
Daisuke
D
Okoshi
Yasushi
Y
Obara
Naoshi
N
Simizu
Seiichi
S
Mukai
Harumi Y
HY
Suzukawa
Kazumi
K
Hasegawa
Yuichi
Y
Kojima
Hiroshi
H
Nagasawa
Toshiro
T
Chiba
Shigeru
S
D051722:ADAM Proteins / Q000037:antagonists & inhibitors / Q000097:blood; D000071120:ADAMTS13 Protein; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D015415:Biomarkers / Q000097:blood; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000628:therapy*; D012074:Remission Induction; D000069283:Rituximab; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D015415:Biomarkers; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human