Meningitis, spondylodiscitis, pneumonia and septic shock with Streptococcus pneumoniae in a previously healthy woman with isolated IgG2-, IgG3-, IgA-deficiency and monoclonal gammopathy of undetermined significance

A 66 years old Caucasian woman with pneumococcal meningitis was treated and discharged after an uncomplicated course. Five months later she was readmitted withfever and right side abdominal pain and diagnosed with pneumococcal spondylodiscitis. One year later she was treated fora severe chest X-ray confirmed left lobar pneumonia. Two years later she was diagnosed with a pneumococcal pneumonia inher left lung with septic shock. An immunedeficiency screen revealed slightly reduced IgA levels, low IgG2 levels, low IgG3 levels and high IgG1 levels. No other immunedefects were identified. She did not respondserologically on vaccination with 13-valentconjugate and 23-valent polysaccharide pneumococcal vaccines. Further evaluations revealed a positive M-component inher blood and a bone marrow biopsy diagnosed her to have monoclonal gammopathy of undetermined significance. To protecther against future life threatening pneumococcal infections she was started on treatment with intravenous immunoglobulin. The case report illustrates the importance of thorough evaluation of patients with unusual infectious disease entities or unusual frequency of infections in individual patients. To optimize prophylactic measures and active treatment options in the individual patient, it is important to identify underlying causes of diseases and immune deficiencies that potentially can lead to life threatening infections. This is illustrated inour case by an undiagnosed monoclonal gammopathy of undetermined significancein an apparently healthy woman with atleast three life threatening documented pneumococcal infections in a two-year period and poor pneumococcal vaccine respons