Fatal lymphoproliferative disease as a complication of Evans syndrome.
A 9-month-old boy had bruising and petechiae. Investigation revealed a Coombs-positive hemolytic anemia and immune-mediated thrombocytopenia. The infant was treated with intravenous immunoglobulin and steroids. The infant eventually had recurrent fevers, hepatosplenomegaly, pulmonary nodules, and parenchymal central nervous system (CNS) lesions develop. Results of a lung biopsy revealed a polyclonal lymphoproliferative disease. Polymerase chain reaction analysis showed the presence of the Epstein-Barr (EB) viral genome in the lung nodules. The infant died from progressive lung disease 6 months after the initial symptoms of Evans syndrome. Lymphoproliferative disease is known to occur in a variety of settings after immunosuppression, especially in solid organ transplant recipients. We report a case of polyclonal lymphocyte proliferation in a patient with Evans syndrome.
Jasty
R
R
Department of Pediatrics, Medical College of Ohio, Toledo, USA.
Strouse
P J
PJ
Castle
V P
VP
D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications*; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D006801:Humans; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000209:etiology*; D008297:Male; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications*; D013577:Syndrome