title: Successful treatment with rituximab of refractory idiopathic thrombocytopenic purpura in a patient with Kabuki syndrome.
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.
authors: Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
authors: Yuka
authors: Y
authors: Torii
authors: Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
authors: Hiroshi
authors: H
authors: Yagasaki
authors: Department of Pediatrics, Komaki City Hospital, Komaki, Japan.
authors: Hidenori
authors: H
authors: Tanaka
authors: Department of Pediatrics, Aichi Prefectural Colony Central Hospital, Kasugai, Japan.
authors: Seiji
authors: S
authors: Mizuno
authors: Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
authors: Nobuhiro
authors: N
authors: Nishio
authors: Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
authors: Hideki
authors: H
authors: Muramatsu
authors: Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
authors: Asahito
authors: A
authors: Hama
authors: Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
authors: Yoshiyuki
authors: Y
authors: Takahashi
authors: Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan. kojimas@med.nagoya-u.ac.jp.
authors: Seiji
authors: S
authors: Kojima
mesh_terms: D000015:Abnormalities, Multiple / Q000097:blood / Q000276:immunology*; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D002675:Child, Preschool; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage*; D008297:Male; D010976:Platelet Count; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000188:drug therapy* / Q000276:immunology*; D000069283:Rituximab; D013577:Syndrome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab