An CD19 Deficient B Cell Abnormality and Selective isotype IgM deficiency in A Malay Child . A Case Report

摘要

Cases of CD19 deficient B cell with hypogammaglobulinemia are rarely reported. We report a 11 year Malay boy with CD19 deficient B cell abnormality . He had recurrent infections including two episodes of chickenpox , repeated pneumoniae noted to be bronchiectatic by age 6 years.His laboratory data showed hypogammaglobulinemis selective for IgM isotype .However B cell showed CD19- but CD20+ phenotype. Mutational analysis revealed an abnormality of exon 1 of the CD19 gene. He had impaired specifc IgG response to pneumococcal polysaccharide antigen . The findings is consistent with diagnosis of CD19 negative B cell with antibody deficiency of the IgM isotype . Regular intravenous Immunoglobulin administration was instituted with improvement clinically.