Age: 16.0
Sex: M
Vaccine: PPV, TD
Symptoms: Antinuclear antibody positive, Immune system disorder
Clinical narrative: It was reported in a published article that a 16 year old male developed a common variable immunodeficiency and negative antibodies after being vaccinated with pneumococcal vaccine 23 polyvalent. The literature article is as followed: A 16 year old boy presented with a 5 year history of recurrent sinopulmonary infections and conjunctivitis. The past history was remarkable for recurrent otitis media in early childhood requiring pressure-equalizing tube placement and adenoidectomy at the age of 3 years. Tonsillectomy was also performed at the age of 10 years because of recurrent tonsillitis. He acquired chicken pox at the age of 3 years and had allergic rhinitis and airway hyperractivity documented by pulmonary function testing. The family history was significant for a sibling with asthma; the patient's mohter had hypothyroidism, sarcoidosis, allergic rhinitis, and breast cancer, and his father had ankylosing spondylitis. The maternal grandfather had asthma and vitiligo. The physical examination was unremarkable, but prominent adenoids were documented by a lateral neck radiograph. Initial lab investigations showed IgG 445 mg/dL (700 to 1800), IgM 82 mg/ dL (60 to 290), and IgA <7 mg/dL (52 to 192). IgG subclasses evaluation showed igG1 268 mg/dL (422 to 1292), IgG2 38 mg/dL (117 to 797), IgG3 7mg/dL (41 to 129), and IgG4 < 0.5 mg/dL (1 to 120). He had no response to pneumococcal vaccine, but excellent titers to Td vaccine (1 to 6461 and 1 to 729 to tetanus and diphtheria, respectively). Five years later, as part of monitoring the immune response, the Td titers were obtained. These were 1 to 3 to both tetanus and diphtheria, with no increase after vaccination. Anti-IgA (IgG) antibodies evaluated by EIA were present. The patient had a normal CBC, lymphocyte subsets by flow cytometry, chest radiograph, and pulmonary function test, and he had a normal DTH response to Candida and mumps antigens. Five years after the initial evaluation, IVIG was started at 400 mg/kg every 4 weeks because of the increasing number of infections, the hypogammaglobulinemia, and the poor response to both Td and Pneumovax. He has been having good trough serum IgG levels (> 500 mg/dL) with no complications and significant improvement in the number of infections. The authors' discussion is as followed: It is known that there is a sequential acquisition of antibody synthesizing capacity in humans. Antibody responses to complex protein (T cell-dependent) antigens such as tetanus and diphtheria toxoid are observed soon after birth, whereas responses to carbohydrate antigens such as the polysaccharides of pneumococci (which do not require cognate T cell help) are not acquired until several years of age. Patients with CVID appear to have a loss of normal antibody synthesis that coincides with declining levels of Igs, particularly IgG and IgA. In our patients, the antibody response to polysaccharide antigens is lost first, and the loss of ability to synthesize antibody against protein antigen comes later, if at all. The response to polysaccharide antigens was either absent when these patients were initially seen or was lost subsequently. Absence of antibody response to polysaccharide antigens may be a universal finding in CVID patients, whereas preservation of T cell-dependent protein antibody response may been seen. It can be seen from the findings above that vigorous response to T cell-dependent protein antigens may be preserved even in the face of profound hypogammaglobulinemia (patient 1). However, even this response may be ultimately lost in some CVID patients (patient 3). The Ig loss was progressive in the three patient's who were followed prospectively; they were either normal or moderately hypogammaglobulinemic at the time of presentation, then progressed to severe hypogammaglobulinemia with an average loss of serum IgG of 120 mg/dL each year. IgG subclasses are not routinely performed on our immunodeficiency patients because the decision to start IVIG depends on the antibody response rather than on the IgG subclasses level. IVIG infusions may be associated with adverse reactions of variable severity. These reactions are usually mild and characterized by back pain, headache, flushing, chills, and myalgia. Severe anaphylactic reactions are rare and sometimes caused by anti-IgA antibodies. These antibodies are usually of the IgG class, although IgM and IgE anti-IgA antibodies have also been reported. Upon internal review, common variable disease was determined to be an other important medical event (OMIC). The article also discussed the experience of three other patients after being vaccinated with pneumococcal vaccine 23 polyvalent (WAES 0302USA02696, 0302USA02834, 0302USA02836).