Age: 9.0
Sex: F
Vaccine: PPV
Symptoms: Nasal oedema, Otorrhoea, Pharyngeal exudate, Respiratory syncytial virus test negative, Rhinorrhoea
Clinical narrative: This report is regarding a 9-year-old girl with a history of recurrent otitis media 15 months of age, chronic sinusitis, and 2 episodes of pneumonia presents for the evaluation of a possible immune deficiency. According to the parents, the patient was always chronically ill and had received multiple courses of antibiotics, including a tria of prophylactic antibiotics. The past history included symptoms consistent with asthma, for which she was being treated with a leukotriene modifier, and allergic rhinitis, for which she was being treated with topical nasal steroids and antihistamines. Family history was positive for allergic disease in both parents. Allergy skin test results are negative for inhalant allergens. There was no family history of an immune deficiency disorder. On physical examination, the patient had thickened tympanic membranes with scars but a good light reflex and were mobile. She had swollen nasal turbinates with white-yellow mucus discharge. There were postpharyngeal exudates and 21 cobblestoning in the posterior oropharynx. Cervical lymph nodes were palpable. The chest examination showed good aeration with no wheezing or rhonchi. There was no hepatosplenomegaly. The remaining physical examination was unremarkable. Laboratory tests included spirometry that showed a FEV1 of 91% of predicted value and a forced expiratory flow at 25% to 75% of forced vital capacity of 87% of predicted value with a normal flow-volume loop. There were no changes with the administration of a bronchodilator. Sinus computed tomography (CT) showed mucosal thickening of the maxillary and ethmoid sinuses but no air-fluid levels. A chest radiograph was normal. Results of a complete blood count with differential were normal. Serum quantitative immunoglobulin levels were as follows: IgG, 522 mg/dL; IgA, 26 mg/dL; and IgM, 27 mg/dL. Specific antibodies to tetanus were present. Antibodies to Mycoplasma species, respiratory syncytial virus, influenza A and B, rubella, and measles were absent. She received PREVNAR 8 weeks before this blood work. For specific pneumococcal antibodies, 2 of 12 serotypes tested were positive. She had a 1:2 titer of anti-B isohemagglutinins (blood type A). There were normal numbers of CD3, CD4, CD8, CD19, and CD56 cells, as determined by means of flow cytometry. Total hemolytic complement was normal. On an unspecified date, she was immunized with PNEUMOVAX23, and 4 weeks later, repeat pneumococcal antibody titers were obtained. Only 5 of 12 pneumococcal polysaccharide antigen specific antibodies were positive (>1.3 mg/mL) and only 4 of the 12 serotypes showed a 2-fold increase. The significant history of recurrent infections, ongoing chronic sinusitis, multiple antibiotic use, poor response to influenza and PNEUMOVAX23, vaccines, and month of presentation (November) led to a consideration of starting replacement intravenous immunoglobulin (IVIG) therapy at 400 mg/kg on a monthly regimen. She did well on the replacement IVIG therapy, with markedly less episodes of otitis media and also improvement in the frequency of sinusitis. Shortly after her parents divorced, the patient moved out of town with her mother. She was lost to follow-up and apparently discontinued her replacement immunoglobulin therapy. At age 16 years, the patient decided to live with her father again and presented with recurrent infections, including draining ears, chronic cough, and recurrent sinusitis. High-resolution chest CT showed no bronchiectasis, and spirometric results were normal. Repeat immune evaluation at age 16 years after being off immunoglobulin replacement therapy for 3 years showed a serum IgG level of 376 mg/dL, IgA level of 7 mg/dL, and IgM level of 21 mg/dL. She had no detectable specific antibodies to 23 pneumococcal polysaccharide antigen serotypes. Her tetanus antitoxoid antibody level was 0.18 IU/mL. Specific antibodies to influenza type A/B and Mycoplasma species were absent. Switched memory B cells were 0.8%. She was given both PREVNAR and PNEUMOVAX23 immunization. After immunization, 4 of the 23 pneumococcal antigen serotypes became positive (>1.3 mg/dL), and 6 increased by more than 2-fold. After tetanus toxoid vaccine, the titer increased to only 0.9 IU/mL; she also responded poorly to influenza vaccine with titers of only 1:4 for influenza A. She was restarted on replacement immunoglobulin therapy in the hospital infusion center at 600 mg/kg per month, with a subsequent follow-up serum IgG level of 840 mg/dL. She wanted to receive IVIG at home and set up with a home-care infusion company. Because of insurance formulary regulations, she was switched to another IVIG product. The patient began to have issues with headaches and myalgias and, on 1 occasion, urticaria after infusion. The only other IVIG option was a lyophilized immunoglobulin product. After discussions with the patient and the father, it was suggested that she switch to a subcutaneous immunoglobulin (SCIG) product. However, as a teenager, she was concerned about her body image and refused to switch. With continued side effects from the intravenous route, the patient finally agreed to switch to the subcutaneous route after discussions with 2 other patients receiving SCIG, one of whom was a young female patient in college. She was started on 600 mg/kg divided weekly, with each infusion given at 4 sites. She did well with no infusion-related headaches or myalgia. A repeat serum IgG level was 982 mg/dL. Additional information is not expected.