Successful Management of Neutropenia in a Patient with CD40 Ligand Deficiency by Immunoglobulin Replacement Therapy

Hyper-igm syndromes are characterized by profound reduction of serum igg, iga, andige levels with normal or increased concentrations of serum igm. cd40 ligand deficiency isx-linked form of the disease, which results in a lack of immunoglobulin class switching fromigm to igg in b cells. in addition to the recurrent infections, a number of patients sufferfrom neutropenia. there are some evidences indicating the effect of g-csf in combinationwith intravenous immunoglobulin (lvig) in improvement of neutrophil counts, which hasbecome the most common procedure to control neutropenia.in this report wc present a 6 year-old patient of cd40 ligand deficiency, who sufferedfrom chronic, severe neutropenia. administration of ivtg was started for him when thediagnosis was made at the agc of 1.5 years and he was on the regular ivig therapy after thattime until! now for a period of 4.5 years. ivig and prophylactic antibiotic therapy, despitecessation of granulocyte colony-stimulating factor, injection after one month, corrected thesevere neutropenic state of this patient.it seems that regular administration of sufficient doses of ivig can be useful in themanagement of neutropenia in cd40 ligand deficiency, which results in better quality of lifewith decreasing occurrence of infection.