title: Successful treatment of autoimmune and lymphoproliferative complications of patients with intrinsic B-cell immunodeficiencies with Rituximab.
abstract: The heterogeneous group of primary immunodeficiencies requires personalized diagnosis and therapy to acheive an optimal outcome for each patient. This was exemplified by two patients with intrinsic B-cell class-switch defects (subclass of Hyper-IgM syndromes), where lymphoproliferation and autoimmunity determined the clinical course for many years due to lack of exact diagnosis. Based on genetics or a novel functional diagnostic approach, a definite individual diagnosis was established for each patient and they started Rituximab therapy. Autoimmune phenomena and generalized lymphadenopathy disappeared and remained well controlled during the observation period (3-4 years) without adverse effects. Quality of life increased remarkably in both patients.
authors.affiliation: Department of Paediatric Pneumology and Neonatology, Hannover Medical School, Carl-Neuberg-Strasse 1, OE6710, 30659 Hannover, Germany. Hennig.christian@mh-hannover.de
authors.forename: Christian
authors.initials: C
authors.lastname: Hennig
authors.forename: Ulrich
authors.initials: U
authors.lastname: Baumann
authors.forename: Claudia
authors.initials: C
authors.lastname: Ilginus
authors.forename: Gerd
authors.initials: G
authors.lastname: Horneff
authors.forename: Juergen
authors.initials: J
authors.lastname: Foell
authors.forename: Gesine
authors.initials: G
authors.lastname: Hansen
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D053306:Hyper-IgM Immunodeficiency Syndrome / Q000188:drug therapy* / Q000276:immunology; D007155:Immunologic Factors / Q000627:therapeutic use*; D008213:Lymphocyte Activation; D008232:Lymphoproliferative Disorders / Q000188:drug therapy* / Q000276:immunology; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab