Sex: F
Vaccine: MMRV
Symptoms: Flow cytometry, Gene sequencing, Immunoglobulins normal, Interleukin level increased, Interleukin-2 receptor increased
Clinical narrative: absent specific antibody response to all vaccines tested; This literature marketed report has been received from the authors of a published literature article, titled as stated below, referring to a 10-year-old female patient.  The patient's concurrent conditions included cherubism and chronic sinusitis with constant nasal congestion. Medical history included severe diarrhea and anal fissures from ages 6 months to 1 year, which resolved spontaneously, recurrent otitis media (as a young child), requiring bilateral myringotomy and tympanostomy tube insertion, speech delay, several episodes of pneumonia, a recurring blistering rash, granuloma annulare diagnosed at age 7, and intermittent hives on the arms and face accompanied by flushing, which appeared to be triggered by temperature changes. Her concomitant vaccines included tetanus vaccine (unspecified) and pneumococcal 13 valent vaccine.  On an unknown date, the patient was vaccinated with measles, mumps, rubella and varicella (oka-merck) virus vaccine live (manufacturer unknown) (dose, route of administration, anatomical location, lot# and expiry date were not reported), administered for prophylaxis.  On an unknown date, the patient sought care for worsening cheek swelling. Physical examination was notable for bilateral maxillary and mandibular fullness and right proptosis. Computed tomographic imaging of the paranasal sinuses showed expansile soft tissue and lucent lesions in the bilateral maxillary bone and bilateral angles of the mandible suggestive of cherubism. One of the lesions was also noted to extend into the floor of the right orbit. Serum immunoglobulin levels were normal. Lymphocyte phenotyping showed normal T-cell numbers and mild B-cell lymphopenia. Antibody levels for Streptococcus pneumoniae showed protection against 3 of 13 serotypes 7 years after receipt of conjugated pneumococcal vaccine. The patient was revaccinated, and after revaccination, the patient developed immunity to 9 of 13 serotypes. Tetanus immunoglobulin G (IgG) was 0.2 IU/mL (reference range: less than 0.1 not protective), measles IgG was less than 13.50 AU/mL, mumps IgG was less than 9 AU/mL, rubella IgG was 26.50 (index), and varicella zoster vaccine response showed more than 4000.00 (index). Natural killer (NK) cells were 0.9 lytic units (minimally reduced). Serum inflammatory cytokine/chemokine levels were measured, showing the following results: soluble interleukin-2 (IL-2) receptor level was 1366.9 pg/mL (mildly elevated), IL-6 was 4.1 pg/mL (mildly elevated), IL-10 was 17.1 pg/mL (mildly elevated), chemokine (C-X-C motif) ligand 9 (CXCL9) was 965 pg/mL (mildly elevated), and IL-18 was 592 pg/mL (mildly elevated). Targeted single gene sequencing for SH3BP2 showed no variants of significance. Trio exome sequencing showed a de novo variant of unknown significance in the phospholipase C gamma 2 (PLCG2) gene. Flow cytometric measurement of lymphocyte calcium flux after surface receptor activation showed markedly enhanced B-cell calcium flux with relatively normal T-cell calcium flux.   The outcome of the event of vaccine induced antibody absent was not reported.  The authors considered the event of vaccine induced antibody absent as related to suspect vaccine.  A copy of the published article is attached as further documentation of the patient's experience.  This is one of two reports referring to the same literature article.  Literature Report:   Lot # is being requested and will be submitted if received.; Sender's Comments:  US-009507513-2412USA005170: