Age: 6.0
Sex: M
Vaccine: VARCEL
Symptoms: Acute lymphocytic leukaemia, Alanine aminotransferase increased, Aspartate aminotransferase increased, Drug ineffective, Eye disorder
Clinical narrative: It was reported in a published article that a six year old white male was vaccinated at 4 years of age with a dose of varicella virus vaccine live. Twenty seven months post-vaccination the patient was diagnosed with T-cell acute lymphoblastic leukemia (ALL), at age 6 years. VZV immunoglobulin G (IgG) titer was positive at the time of diagnosis of ALL. At the age of 7, the patient, who's T-cell ALL was in remission, presented to the oncology clinic with complaints of high fever (104 degrees F) and an exanthematous vesicular rash on the forehead, trunk, and lower extremities. There was no history of visual complaints, cough, difficulty breathing, abdominal pain, or jaundice. A clinical diagnosis of chicken pox was made and the patient was admitted to the hospital for further evaluation and antiviral therapy. He had received his last course of chemotherapy (vincristine, prednisone, 6-mercaptopurine, and methotrexate) three weeks prior to presentation. Two weeks prior to presentation he was exposed to a child with chicken pox at school. He did not receive post-exposure prophylaxis. He had not previously experienced chicken pox. Physical exam revealed a non-toxic male with temperature 103.4 degrees F, heart rate 136 beats/min, respiratory rate 22 breaths/minute, and blood pressure 104/61 mmHg. Examination of the skin was remarkable for cutaneous lesions on an erythematous base in various stages from papules to vesicles to scabbed vesicles located on the forehead, trunk, and lower extremities, consistent with chicken pox. The rest of the physical examination was unremarkable. Laboratory data showed a hemoglobin of 8g/dl, platelet count of 112,000 cells/mm3, and a white blood cell count of 2900 cells/mm3 with 82% neutrophils, 11% bands, and 7% lymphocytes. Liver enzymes were mildly elevated at aspartate aminotransferase (AST), 301 U/L (normal range 15-50 U/L) and alanine aminotransferase (ALT), 494 U/L (normal range 15-50 U/L). Blood urea nitrogen, serum creatinine, and bilirubin levels were normal. Polymerase chain reaction (PCR) assay on the blood specimen was negative for VZV DNA. Serum VZV IgG titer was positive. He received IV acyclovir, 500mg/m squared/dose q 8 hr for 5 days, followed by 9 days of oral acyclovir (500mg/dose q 6 hr), and his rash gradually resolved. At the time of discharge, he was afebrile, all skin lesions had crusted, and liver enzyme levels had decreased (AST, 41 U/L, and ALT 128 U/L). Two weeks following chicken pox, he presented to the oncology clinic with a 1-day complaint of blurred vision in his right eye. Physical exam revealed a well-appearing male with normal vital signs. No new skin lesions were present. On ophthalmologic examination, his visual acuity was 20/30 OD and 20/20 OS. His left eye was normal. A right afferent pupillary defect was present and visual field testing showed a superior temporal defect. The anterior segment was normal, but there was 1+ cells in the vitreous. Funduscopic examination of the right eye revealed a focus of hemorrhagic retinitis in the inferotemportal quadrant. Cerebrospinal fluid analysis was normal. Contrast-enhanced computed tomography scan and magnetic resonance imaging study of the head and orbits were unremarkable. A bone marrow aspirate was normal. In view of the onset of ocular symptoms and signs following the appearance of the typical VZV cutaneous lesions which had followed recent exposure to VZV, a presumptive diagnosis of varicella retinitis was made. He was admitted and started on IV acyclovir (500 mg/m squared/dose q 8 hr). Treatment was successful in preventing progression of the retinitis. Serial ophthalmologic evaluation over the next 2 months showed gradual resolution of inflammatory changes in the right eye. He received IV acyclovir for 4 weeks, followed by a 12 week course of oral therapy (400 mg/dose q 6 hr). He also received prednisolone 1% ophthalmic drops and scopolamine ophthalmic drops to the right eye for 12 weeks. His chemotherapy was held temporarily during this period. When last seen at follow up, 3 months after onset of retinitis, visual acuity was 20/25 OD and 20/20 OS. A residual scar remained in the area of previous retinitis. The left eye was not affected. The patient's retinitis was successfully treated with prolonged sequential parenteral/oral acyclovir. Virus isolation from the skin lesions was not attempted, so strain type information was not available. Additional information has been requested. The article also discussed the experience of a 9 year old male while on therapy with varicella virus vaccine live (WAES0303USA00475). A copy of the published article is attached as further documentation of the patient's experience.