Age: 1.1
Sex: F
Vaccine: HEPA, MMR, VARCEL
Symptoms: Lymphoblast count, Lymphocyte count decreased, Lymphopenia, Ophthalmic herpes zoster, Ophthalmological examination abnormal
Clinical narrative: Information has been received from the authors in a literature article concerning a patient with VARIVAX (Merck) (manufacturer unknown), M-M-R II and methylprednisolone (manufacturer unknown) therapy. At 13 months of age, a previous healthy girl with RAG2 deficiency presented acutely with fever, jaundice, cough, rhinorrhea, diarrhea and approximately 10 erythematous papules on her torso and extremities. Preliminary studies revealed direct Coombs positive anemia and leukocytosis with granulocytes predominating. Initial treatment measures included blood transfusions, intravenous immunoglobulin (MG) and high dose methylprednisolone (manufacturer unknown) (up to 80 mg/day), leading to transient resolution of hemolysis. In addition, she developed autoimmune thrombocytopenia (nadir 25,000 platelets/1/4.L). Three weeks after onset of illness, her rash evolved into discrete 3-mm eschars; however, very few, if any new skin lesions developed. Skin biopsy revealed intranuclear inclusion bodies typical of Herpesviridae and intravenous acyclovir was initiated. Despite antiviral therapy, which later included foscarnet, her clinical status deteriorated over subsequent weeks. Obtundation ensued, and the patient died due to complications of hepatic and renal failure, disseminated intravascular coagulation and recalcitrant autoimmune hemolytic anemia. Three weeks before onset of illness, the patient received her first VARIVAX (Merck) (manufacturer unknown) (dose and route unknown), MMR (manufacturer unknown) (dose and route unknown) and hepatitis A (manufacturer unknown). There was no obvious contraindication to the administration of live virus vaccination. She had previously received all vaccinations as per the Advisory Committee on Immunization Practices (ACIP) recommended schedule, including the rotavirus series, which were well tolerated. Prior to death, vaccine strain varicella zoster virus (VZV) was detected in her cerebrospinal fluid (723 copies/mL), skin (137,156 copies/mL) and esophagus by PCR assays and immunohistochemistry staining, respectively. Throat swabs, also obtained prior to death, detected mumps and rubella by PCR assays. Stool samples submitted for viral electron microscopy were negative, including assessment for rotavirus. Ophthalmic examination revealed corneal dendrites with typical features of VZV infection. Autopsy demonstrated VZV dissemination in the lungs and liver as well as profound lymphoid depletion of lymph nodes and thymic tissue. VZV, mumps and rubella isolated from the patient were confined as vaccine genotypes Oka, Jeryl-Lynn and RA 27/3, respectively. The reporter considered the events to be related to VARIVAX (Merck)(manufacturer unknown), MMR (manufacturer unknown), methylprednisolone (manufacturer unknown) and hepatitis A (manufacturer unknown). Upon internal review, renal failure, hepatic failure and disseminated intravascular coagulation were considered to be medically significant. Additional information has been requested.