Age: 11.0
Sex: F
Vaccine: VARCEL
Symptoms: Drug ineffective, Hypoxia, Infection, Pneumonitis, Rash vesicular
Clinical narrative: Information has been received from a physician concerning an 11 year old female pt who was vaccinated with varicella virus vaccine live and approx. 5 weeks, post vax, presented with a diffuse vesicular rash and respiratory distress requiring hospitalization and intubation. The pt was dx'd with "post vaccine varicella pneumonitis". A respiratory aspirate, as well as BAL fluid specimens were both found to be positive for VZV through the hospital laboratory, however, the physician stated that a number of cultures had been "contaminated with bacteria". The pt is currently improving on acyclovir and is undergoing an "immune work-up". The physician indicated as of 12/12/00, the pt had been extubated. The physician is interested in submitting specimens to a university for PCR analysis. No further information is available. 15-Day follow-up received on 2/7/01 provided no additional data. The follow up received on 7/23/03 indicated that both the bronchioalveolar lavage and the endotracheal secretions specimen were VZV (+). Both specimens were contaminated with bacteria. The presence of Oka/Vaccine VZV was identified by PCR analysis. Further follow-up info from a physician indicated suspicion that the pt has cystic fibrosis with a positive sweat test. "The other reason this was suspected was that the bronchial secretions had an incrreased bacteria load which lead the lab to pursue PCR since they were not able to grow VZV." The physician indicated that "the pt is undergoing an extensive immunologic evaluation." Follow-up info from a physician for the case of hte 11 year old female which indicated that he believes he has discovered a "novel" immune deficiency. Subsequently, the patient's experience was reported in a published article. The patient had recurrent and presumably virus respiratory infections and mental retardation and presented with a 5 day history of an erythematous rash over her trunk and scalp and with diminished activity. Three days before presentation she developed labored breathing, cough and increased respiratory secretions. on the day of admission, she was lethargic, hypothermic, hypoxemic, and had several erythematous papulovesicular lesions over her turnk. Results of the lab studies performed when she was admitted were notable for providing evidence of leukopenia, thrombocytopenia, and elevated serum alanine aminotransferase. Radiography of the patient's chest revealed bilateral symmetric alveolar infiltrates. The patient was admitted to the ICU, and mechanical ventilation was administered. IV acyclovir and methylprednisolone were also admitted. The patient was extubated on hospital day 6 and was discharged on hospital day 17, to complete a 21 day course of acyclovir with follow up at an immunology clinic. It was noted that he patient died 10 months after the reported illness, presumably because of the embolic complications related to a  closed fracture of the femur. Results of the analysis of skin lesion smears were positive for VZV, by indirect immunofluorescent assay. An endotracheal tube-aspirate sample was subjected to RFLP analysis and was identified as the Oka vaccine strain. Assessment of the patient's immune system revealed a normal phagocytic and complement function as well as IgG, IgA, and IgM levels that were normal to high, both during her acute illness and recovery. It was noteworthy that the patient had markedly elevated titers of antibodies against several Herpes viridae: CMV specific IgG was > 14 IU/mL but IgM was absent; Epstein Barr virus specific IgG was >19 IU/mL and herpes simplex virus-specific IgG was 1.08 IU/mL. During the patient's acute infection, VZV specific IgG was elevated to 7.51 IU/mL, indicating VZV specific immunity. It was noted that VZV specific lymphocyte proliferation measured 5 months after the patient's disseminated VZV infection revealed appropriate virus specific proliferation. The patient was negative for HIV-1 and -2, by antibody testing, RT PCR for HIV RNA, and culture. Lymphocyte analysis revealed a low to normal absolute lymphocute count during our patient's illness, with an inverted CD4-CD8 ratio that normalized 1 month later. It was noted that the levels of the patient's NKT cells, as measured on the basis of either CD16/CD56/CD3 or CD161/CD3, were abnormally low. Multiple analyses of the patient's PBMC's by flow cytometry revealed a deficiency of CD16/CD56/CD161/CD3 NKT cells in general and a profound deficiency of Valpha24 iNKT cells in particular. It was noted that her immunodeficiency had presented only as a recurrent upper respiratory tract infections; no mention was made of past measles immunization, and she may not have had difficulties with the vaccine. No further info is available.