Age: 1.03
Sex: M
Vaccine: VARCEL
Symptoms: Alanine aminotransferase increased, Aspartate aminotransferase increased, Infection, Pyrexia, Rash maculo-papular
Clinical narrative: Information has been received from a physician concerning a 14 month old male with asthma, no known drug allergies, no history of chicken pox exposure to chicken pox or shingles, and a social history significant in that his parents were in an automobile accident in June 2003 in which his father died and his mother became paralyzed. The family member now caring for the child found his vaccination history was not complete and went forward with the necessary vaccinations. On 8/18/03 the pt was vaccinated in the left thigh with a first dose of varicella virus vaccine live (641148/0791M). Concomitant medications included albuterol nebulizer prn and ofloxacin gtts, both ears. On 9/5/03 the pt developed a vesicular rash which progressed to involve his entire body over the next 3-4 weeks. It was described as a diffuse maculopapular rash with hundreds of lesions in multiple different stages, including vesicles and scabbing consistent with VZV. The lesions were distributed diffusely from head to toe with noted oropharyngeal lesions. His maximum temp was 102.6. On 10/10/03 he was admitted to a hospital due to the persistence of the rash. He was begun on IV acyclovir at that time. On 10/10/03, while on parenteral antiviral therapy, his AST and ALT were normal. On 10/15/03 they were 5x normal. The pt had a punch biopsy done which was negative for HSV1 and HSV2. The punch biopsy did reveal multi-nucleated giant cells consistent with a VZV infection (also reported as a lesion biopsy that was PCR-positive for VZV DNA). On the evening of 10/16/03 or the day of 10/17/03 the pt was transferred to a different hospital with a disseminated rash, fevers and an elevated AST and ALT. On the weekend of 10/18/03 the pt was seen by the physician. His IV acyclovir was increased to 60mg/kg/day (high dose). An abdominal sonogram has shown "infiltrated processes" in the liver which is most likely evidence of visceral dissemination. The AST and ALT remained elevated as of 10/19/03. On 10/20/03 it was reported that the pt's AST and ALT had begun to decrease over the last 2 days and that the child was improving. It was reported that the pt's immune work-up has been quite extensive to date, and is all "normal" so far. However, it was also noted that it is suspected that the pt may have familial erythrophagocytic lymphohistiocytosis. This has not been confirmed and the pt was continuing to be assessed for an immunodeficiency. On 10/21/03 a scab from a lesions was obtained and sent to the lab for VZV identification. It was also reported that on an unknown date the pt was vaccinated with a dose of MMR Ii (645405/0132N), a dose of Hib conj vaccine (2A744), a dose of hep B virus vaccine, a dose of poliovirus vaccine inactivated and a dose of diphtheria toxoid +pertussis vaccine+tetanus toxoid. A lot number of 21882C2 was noted but not indicated for which vaccine. Additional info has been requested.  Follow up on 11/25/2003: "On 10/21/2003 a scab from a lesion was obtained and sent to the laboratory for VZV identification.  ON 11/11/2003 it was reported that the preliminary result of the specimen has identified VZV, however it had not yet been typed to identify the strain.  It was also reported that th epatient was improving and was on the "general floor".  His rash had resolved and there were no new lesions.  The patient's liver enzymes had flucuated and had been normalizing but are now beginning to rise again.  It was also noted that a diagnosis of severe combined immunodeficiency rather than familial erythrophagocytic lymphohistiocytosis was being considered.  Additional information has been requested." Follow up on 01/09/04: "It was also reported that on an unknown date the pt was vaccinated with a dose of MMR (Lot # 645405/0132N), a dose of Hib conj vaccine (manufacturer unknown, Lot # 2A744), a dose of hepatitis B virus vaccine rHBsAg (yeast)(manufacturer unknown), a dose of poliovirus vaccine inactivated (unspecified) and a dose of diphtheria toxoid (+) pertussis vaccine (unspecified)(+) tetanus toxiod.  A lot # of 21882C2 was noted but not indicated for which vaccine.  On 12/31/03 it was reported that results of PCR analysis were positive for vaccine strain.  Attempts to obtain follow up information from the physician have been unsuccessful.  Additional information is being sought." On 16 Jan 2004 it was reported by a nurse that no other diagnoses have been made (ie. no immunodeficiency or familial erythrophagocytic lymphohistiocytosis). It was also noted that the nurse thought that "at present" the patient was doing well, but she was "not certain". Additional information is being sought. ON 22Mar2004, follow up information reported by a physician indicated that the pt was noted to have progressive varicella due to the disseminated OKA strain VZV and a serious immunodeficiency. On 22Mar2004 it was also reported by a physician that in January 2004 the pt was hospitalized briefly with a febrile illness and a cough, but he seemed to recover and never required oxygen. On 05Feb04 a killing assay "again" returned abnormal. The pt had markedly elevated intracellular perforin levels although it was not known if the protein was functional. It was noted that the pt had a very junky chest exam and upper respiratory congestion but otherwise seemd Ok on this vist. The varicella antibody was again positive on this visit. The pt's presentation and lab results both indicated that the pt has a serious deficiency in kill and is probably at substantial risk for life threatening viral infections. The pt's workup was ongoing. The physician noted that outside of bone marrow transplant he did not know of any other type of therapy that could be considered in this case. If the pt developed another similar severe infection and survived, he would vote for a bone marrow transplant despite risks because he had doubts the pt's chances for a long term survival otherwise. No further information is available.