Age: 2.0
Sex: F
Vaccine: PPV, UNK
Symptoms: Antibody test negative, Blood albumin normal, Blood immunoglobulin G normal, Vaccination failure
Clinical narrative: Post-vaccination titer levels were concerning for severely impaired antibody response to polysaccharide antigens; Post-vaccination titer levels were concerning for severely impaired antibody response to polysaccharide antigens; Literature Report. This literature marketed report had been received from the authors of a published article, titled as stated above, and referring to a 12-year-old female patient. The patient's medical history included hospitalization in the intensive care unit for the first 5 months of life; the patient was treated for coagulase-negative staphylococcal sepsis at age 3 weeks; pneumonia based on chest computerized tomography (CT) at age 6 weeks; probable sepsis at age 2 months; Sanger sequencing identified homozygous n.51G > A variants in RNU4ATAC, which is a mutation known to be associated with MOPDI/III in a homozygous state; bilateral hip dislocation; tracheostomy; laboratory evaluation at age 18 months revealed normal IgG, low IgA, protective diphtheria and tetanus titers, and decreased numbers of CD19 + B cells and CD3 +, CD4 +, and CD8 + T cell. Lymphocyte proliferativeresponses to PHA and pokeweed were normal; and, at the age of 2 years, the patient was hospitalized for left lower lobe pneumonia based on chest X-ray (CXR). The patient' concurrent conditions inlcuded intra-uterine growth retardation (IUGR); spondyloepimetaphyseal dysplasia with extreme short stature; microcephaly with partial agenesis of corpus callosum; seizure disorder; hemorrhagic stroke; global developmental delay; ventilator dependent status post and is G-tube dependent. She was not taking medications that could lead to decreased production of immunoglobulins. Her concomitant medications included levetiracetam.  On an unknown date, the patients were vaccinated with pneumococcal vaccine, polyvalent (23-valent) (PNEUMOVAX23) injection (reported as pneumococcal polysaccharide vaccine [PPV23]) and pneumococcal vaccine conj 13v (crm197) (reported as 13-valent pneumococcal conjugate vaccine [PCV13]), both administered for prophylaxis (doses, vaccination schemes, frequencies, routes of administration, anatomical sites of injection, lot numbers, and expiration dates were not reported).  On an unknown date, her post-vaccination titer levels were concerning for severely impaired antibody response to polysaccharide antigens (antibody test negative, vaccination failure), though titers were not drawn until approximately 4 months after pneumococcal vaccine, polyvalent (23-valent) (PNEUMOVAX23) administration. The patient received immunizations per routine schedule, including pneumococcal vaccine conj 13v (crm197), yet showed protective response to less than 50 percent of PCV13-serotypes. However, total IgG remained normal. Albumin was normal. At age 3 years, the patient was admitted to the ICU for respiratory failure secondary to RSV pneumonia and hospitalized for 5 months. Immunoglobulin replacement therapy was started during the admission. Since then, she has had 3 episodes of uncomplicated cellulitis treated with oral antibiotics, without other bacterial or severe viral infections. IgA and B and T cell counts gradually normalized after several years. Antibody responses have not been reevaluated as she has continued immunoglobulin replacement therapy. The outcome of these events was not reported.  The causal relationship between antibody test negative and vaccination failure regarding pneumococcal vaccine, polyvalent (23-valent) (PNEUMOVAX23) vaccination was determined as related.  This is one of two reports regarding the same article.  A copy of the published article is attached as further documentation of the patient's experience.