Rituximab induces remission of cerebral ischemia caused by thrombotic thrombocytopenic purpura
Article first published online: 26 FEB 2003
European Journal of Haematology
Volume 70, Issue 3, pages 183–185, March 2003
How to Cite
Tsai, H.-M. and Shulman, K. (2003), Rituximab induces remission of cerebral ischemia caused by thrombotic thrombocytopenic purpura. European Journal of Haematology, 70: 183–185. doi: 10.1034/j.1600-0609.2003.00026.x
- Issue published online: 26 FEB 2003
- Article first published online: 26 FEB 2003
- Accepted for publication 29 December 2002
- thrombotic thrombocytopenic purpura;
- von Willebrand factor;
Objective: This report describes the experience of a case of atypical thrombotic thrombocytopenic purpura (TTP) whose diagnosis was based on severe deficiency of the von Willebrand factor (vWF) cleaving metalloprotease ADAMTS13.
Methods: The level of ADAMTS13 activity, the titer of the inhibitors of this protease and the size distribution of vWF multimers in plasma samples were analysed in a patient with recurrent episodes of dizziness and blurred vision.
Results: In the absence of thrombocytopenia or microangiopathic hemolysis, diagnosis of TTP was established by demonstration of very low ADAMTS13 activity levels and the presence of inhibitors of this protease. After rituximab therapy decreased the inhibitor titer and increased the ADAMTS13 level, the patient has had no relapse of ischemic symptoms in the following 16 months.
Conclusions: Acute neurological deficits may occur in TTP without concurrent thrombocytopenia or microangiopathic hemolysis. The role of rituximab for patients with TTP deserves further exploration.