Sex: M
Vaccine: PPV
Symptoms: Antibody test negative, Blood immunoglobulin A normal, Blood immunoglobulin G normal, Blood immunoglobulin M normal, CD4 lymphocyte percentage decreased
Clinical narrative: postimmunization pneumococcal titers waned from protective; had immune defects; This literature marketed report has been received from the authors of a published article, titled as stated below, and refers to a a fully immunized boy, who at the time of the report was 5-year-old, with Zhu-Tokita-Takenouchi-Kim syndrome (ZTTK) syndrome (confirmed by genetic testing, inheritance: de novo) due to a nonsense mutation in the SON gene (c.3334C more than T). His history was significant for multiple upper respiratory infections, viral gastroenteritis, hand-foot- mouth disease, and recurrent acute otitis media requiring tympanostomy tube placement.  On an unknown date, the patient was vaccinated with pneumococcal 23v polysaccharide vaccine (manufacturer unknown), injection (dose, dose number, route of administration, anatomical location, lot number and expiration date were not reported) for prophylaxis.  On an unknown date (reported as 2.5 years ago), initial postimmunization pneumococcal titer was protective (more than or equal to 1.3 microgram/mL) in 18 of 23 subtypes. On an unknown date, the patient was vaccinated with a booster dose of pneumococcal 23v polysaccharide vaccine (manufacturer unknown), injection (dose, dose number, route of administration, anatomical location, lot number and expiration date were not reported) for prophylaxis. On an unknown date (reported as over 2.5 years), repeat pneumococcal booster titer was protective (more than or equal to 1.3 microgram/mL) in 8 out of 23 subtypes; it waned from 18 out of 23 subtypes (antibody test negative). On an unknown date, the patient presented with recurrent infections. Laboratory evaluation revealed low immunoglobulins (Igs) across all classes (IgG, 461 mg/dL, range: 457-1120 mg/dL; IgM, 35 mg/dL, range: 58-198 mg/dL; IgA, 10 mg/dL, reference: 35-192 mg/dL). On an unknown date, lymphocyte subset studies revealed a reduction in the percentage of CD4+ T-cells to 22.9%, with a normal absolute count of 829 cells/microliter (reference range for age: 28%-47% positive; 700-2200 cells/microliter). The normal absolute values for CD3, CD8, CD19 and CD56 were 2306 cells/microliter (range: 1400-3700 cells/microliter), 1249 cells/microliter (range: 490-1300 cells/microliter), 891 cells/microliter (range: 390-1400 cells/microliter), 547 cells/microliter (range: 126-729 cells/microliter), respectively. It was reported that there was also a reduction in the CD4+-to-CD8+ ratio to 0.66. T mitogens were normal with proliferation to concanavalin A, phytohemagglutinin, and CD3 ligation with 96.7%, 96.55%, and 91.3% positive, respectively. Diphtheria titer was 0.17 IU/mL (range: more than 0.10 IU/mL). On an unknown date, prebooster tetanus titer was 6.61 IU/mL. On an unknown date, postbooster tetanus titer was 3.78 IU/mL (range: more than IU/mL). On an unknown date, the patient was treated with intravenous Ig (IVIg) (400 mg/kg) every 4 weeks for management of specific antibody deficiency. He responded well to this therapy and 1 year later reported that he had not required any antibiotics since starting IVIg. He reported 1 infection with confirmed severe acute respiratory syndrome Coronavirus 2, which was self-limited, lasting less than 5 days. On an unknown date (reported as at his follow-up appointment 2 years later), the patient reported 1 course of antibiotics for streptococcal pharyngitis, associated with only mild symptoms. On an unknown date, repeat IgG, repeat IgM and repeat IgA were 827 mg/dL, 30 mg/dL and less than 15 mg/dL, respectively. The outcome of the event of antibody test negative was not reported.  The causality between the event of antibody test negative and the suspect vaccine was reported as related.  A copy of the published article is attached as further documentation of the patient's experience.  This is one of several reports derived from the same literature article.  Lot number is being requested and will be provided if available.  Sender's Comments:  US-009507513-2408USA000594: