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Rituximab for the treatment of post‐bone marrow transplantation refractory hemolytic anemia in a child with Omenn's syndrome
Author(s) -
Silvana Briuglia,
Antonella La Mazza,
Basilia Piraino,
Trombetta Domenico,
Saija Antonella,
Salpietro Carmelo
Publication year - 2007
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2007.00678.x
Subject(s) - medicine , rituximab , refractory (planetary science) , hemolytic anemia , bone marrow transplantation , anemia , autoimmune hemolytic anemia , transplantation , immunology , surgery , lymphoma , physics , astrobiology
Omenn's syndrome is a rare severe combined immunodeficiency that kills affected subjects before the end of the first year of life unless patients are treated with bone marrow transplantation (BMT). Unfortunately, post‐BMT patients may develop autoimmune diseases, such as autoimmune hemolytic anemia (AIHA), which sometimes fails to respond to standard therapies. Rituximab is a chimeric, human, immunoglobulin G1/k monoclonal antibody specific for the CD20 antigen expressed on the surface of B lymphocytes. Rituximab is currently only labeled for treatment of B‐cell lymphoproliferative disorders, such as B‐cell non‐Hodgkin's lymphoma and follicular lymphoma; however, it is also employed in the treatment of a variety of disorders mediated by auto‐antibodies, such as AIHA and transplant‐related autoimmune disorders. Herein, we describe the case of a 23‐month‐old male child with Omenn's syndrome, who had undergone BMT and was successfully treated with rituximab (375 mg/m 2 intravenously, weekly for three times) for refractory post‐BMT hemolytic anemia. Our findings evidence that rituximab should be considered for treatment of post‐BMT AIHA refractory to traditional therapy also in children with primary immunodeficiencies; furthermore, rituximab might represent a means to obtain remissions without the toxic effects associated with corticosteroid and immunosuppressive agents.