Age: 1.6
Sex: F
Vaccine: PPV
Symptoms: Laboratory test abnormal, Lymphadenitis, Mucosal inflammation, Pneumonia, Pyelonephritis
Clinical narrative: It was reported in a published article that a 19 month old female patient, a product of father/daughter conception, fully immunized as an infant, with interleukin-1 receptor-associated kinase 4 deficiency and a history of frequent bacterial infections and mutliple hospitalizations starting at 5 weeks of age for buccal cellulitis associated with severe thrush and Staphylococcal aureus bacteremia, cervical lymphadenitis, severe impetigo, pneumonia and septicemia, bacterial arthritis, cellulitis and septicemia and pneumonia who at 1.6 years of age was vaccinated with a first dose of pneumococcal 23v polysaccharide vaccine. At 19 months of age, the patient developed cervical lymphadenitis. At 30 months of age, the patient developed lymphadenitis and septicemia. At 3.3 years of age was vaccinated with a second dose of pneumococcal 23v polysaccharide vaccine. At 3.8 years of age was vaccinated with a third dose of pneumococcal 23v polysaccharide vaccine. At 46 months of age, the patient developed pyelonephritis. At 48 months of age, the patient developed colitis. At 49 months of age, the patient developed sinusitis. At 51 months of age, the patient developed mucositis. At 54 months of age, the patient developed sinusitis. At 63 months of age, the patient developed sinusitis. At 86 months of age, the patient developed pneumonia. At 87 months of age, the patient developed colitis. The recurring bacterial and fungal infections required IV immunoglobulin therapy. At 88 months of age, the patient received IVIG due to chronic colitis. Five months after IVIG therapy, at 93 moths of age, the patient developed sinusitis. Pseudomas aeruginosa and Serratia marcescens cultured from the sinuses. The patient was treated with antibiotics and surgical drainage. At 94 to 99 months of age, the patient developed nasal congestion. At the time of the report, at 8 years of age, the patient continued to have severe infections such as pneumonia, septicemia, bacterial arthritis, lymphadenitis, and pyelonephritis, requiring multiple hospitalizations for IV antimicrobial treatment. No fever accompanied any of these infections. Extensive immunologic analyses on several occasions have been normal. Antibody responses to pneumococcal 23v polysaccharide vaccine given at 1.6, 3.3, and 3.8 years of age were tested. The patient responded to 4 to 12 polysaccharide antigens. These responses were short lived, as the titers decreased back to the baseline 5 to 6 months after the second and third vaccinations. Eighteen  months after a fourth immunization with diphtheria and tetanis toxoid, antibodies to diphtheria and tetanus toxoid were undetectable. The patient received a fifth immunization with diphtheria and tetanus toxoid at 3.2 years of age. The responses 1 month after the fifth DT were borderline to normal. Six months after the fifth immunization, antibody titers to both antigens were significantly lower. The patient received immunization with bacteriophage X174. After the primary immunization with bacteriophage X174, the peak Kv was less than 2 SD and showed a poor amplification and rapid decline in titers. The results of the secondary response showed that the peak Kv was significantly less than 2 SD, demonstrating a considerable depressed secondary response. These antibody titers indicated suboptimal antibody response. The secondary immunization produced a predominant IgG isotype, demonstrating normal isotype switching. Booster immunization with diphtheria/tetanus toxoid, pneumococcal 23v polysaccharide vaccine, and bacteriophage X174 demonstrated an impaired antibody responses either to polysaccharide or protein antigens or to a neoantigen bacteriophage. Additional information has been requested. Follow up information was received from the primary author of the published article. The reporter stated the patient did not respond to the pneumococcal 23v polysaccharide vaccine as the patient has an immunodeficiency, i.e. the patient did notmake antibodies. The reporter also stated there was no adverse reaction. Additional information is not expected.