Published in:
01-09-2009 | Case Report
Successful treatment with rituximab of refractory idiopathic thrombocytopenic purpura in a patient with Kabuki syndrome
Authors:
Yuka Torii, Hiroshi Yagasaki, Hidenori Tanaka, Seiji Mizuno, Nobuhiro Nishio, Hideki Muramatsu, Asahito Hama, Yoshiyuki Takahashi, Seiji Kojima
Published in:
International Journal of Hematology
|
Issue 2/2009
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Abstract
Kabuki syndrome (KS) is often associated with autoimmune abnormalities, such as idiopathic thrombocytopenic purpura (ITP), autoimmune hemolytic anemia, leukoplakia and thyroiditis, as well as congenital anomalies. We herein present a KS patient with refractory ITP who achieved durable and complete remission in response to a total of four once-monthly infusions of rituximab. KS patients are often more susceptible to infection, so splenectomy should be avoided. Therefore, rituximab therapy is an alternative option for KS patients with ITP who fail to respond to first-line therapy.