title: Transient effect of anti-CD20 therapy in a child with 22q11.2 deletion syndrome and severe steroid refractory cytopenias: a case report.
abstract: We report on the development of steroid-refractory recurrent cytopenias in a child with 22q11.2 deletion syndrome. His first hematological complication was autoimmune hemolytic anemia at 3 months of age. Thereafter, he developed severe autoimmune cytopenias of all 3 hematological lineages with poor response to steroids and intravenous immunoglobulin. At the age of 2½ years, a course of anti-CD20 therapy (Rituximab) was given with transient hematological recovery. Because of persistent symptoms, bone marrow transplantation from a matched unrelated donor was performed. Although the data in the use of anti-CD20 therapy in children with 22q11.2 deletion syndrome and autoimmune cytopenias are limited, our experience suggests its potential benefit.
authors.affiliation: Second Department of Pediatrics, "P. & A. Kyriakou" Children's Hospital, University of Athens Medical School, Athens, Greece. alsoldat@med.uoa.gr
authors.forename: Alexandra
authors.initials: A
authors.lastname: Soldatou
authors.forename: Theodora
authors.initials: T
authors.lastname: Anastassiou
authors.forename: Olga
authors.initials: O
authors.lastname: Vougiouka
authors.forename: Evgenios
authors.initials: E
authors.lastname: Goussetis
authors.forename: Lydia
authors.initials: L
authors.lastname: Kossiva
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D016026:Bone Marrow Transplantation; D004062:DiGeorge Syndrome / Q000097:blood* / Q000188:drug therapy* / Q000601:surgery; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007231:Infant, Newborn; D008297:Male; D010198:Pancytopenia / Q000097:blood / Q000188:drug therapy* / Q000601:surgery; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab