Three weeks following vaccination for chickenpox (varicella), mumps, measles, rubella and hepatitis A, a 13-month-old previously healthy girl presented to hospital in Denver, Colorado with high fever, cough, runny nose and diarrhea. Her skin and the whites of her eyes had yellowed by jaundice and she also had about 10 small skin lesions on her torso and limbs. A Coombs test revealed that an immune-mediated reaction was destroying her red blood cells and she was positive for anemia and leukocytosis, an increase in white blood cells.
The toddler was treated initially with blood transfusions and intravenous immunoglobulin (IVIG) and high dose steroids which temporarily halted the red blood cell destruction. She developed autoimmune thrombocytopenia — as a result of her immune system attacking her blood platelets. Three weeks after onset of illness, her rash evolved into 3mm spots of sloughing dead tissue though few, if any, new lesions appeared. Skin biopsy suggested Herpes infection and anti-viral medication was begun. Nevertheless, the girl grew iller over the following weeks. She lost consciousness and died due to complications of hepatic and renal failure, extensive blood clotting and recalcitrant autoimmune anemia.
Prior to her 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 taken prior to her death detected mumps and rubella by PCR assays. An autopsy revealed that the vaccine strain varicella virus had spread to her liver and lungs as well.
Testing showed that the girl had RAG mutations — genetic defects that result in imunodeficiency, in this case undetected prior to live virus vaccination which resulted in her death.
J Clin Immunol. 2015 Nov;35(8):754-60. doi: 10.1007/s10875-015-0207-8.