Successful treatment with rituximab of refractory idiopathic thrombocytopenic purpura in a patient with Kabuki syndrome.

TitleSuccessful treatment with rituximab of refractory idiopathic thrombocytopenic purpura in a patient with Kabuki syndrome.
Publication TypeJournal Article
Year of Publication2009
AuthorsTorii Y, Yagasaki H, Tanaka H, Mizuno S, Nishio N, Muramatsu H, Hama A, Takahashi Y, Kojima S
JournalInternational journal of hematology
Volume90
Issue2
Pagination174-6
Date Published2009 Sep
ISSN1865-3774
KeywordsAbnormalities, Multiple, Antibodies, Monoclonal, Antibodies, Monoclonal, Murine-Derived, Child, Preschool, Humans, Immunologic Factors, Male, Platelet Count, Purpura, Thrombocytopenic, Idiopathic, Syndrome
Abstract

Kabuki syndrome (KS) is often associated with autoimmune abnormalities, such as idiopathici thrombocytopenic purpurai (ITPi), autoimmune hemolytic anemiai, leukoplakia and thyroiditis, as well as congenitali 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.

Alternate JournalInt. J. Hematol.
Citation Key393